2026
Advances in cystic fibrosis: CFTR modulator triple combinations.
Authors: Burgel PR, Mall MA.Eur Respir J. 2026 Jan 8;67(1):2501783. doi: 10.1183/13993003.01783-2025. Print 2026 Jan.PMID: 41309268 Free PMC article.
Clinical characteristics and outcomes in the adult cystic fibrosis population in Europe from 2014 to 2024: analysis of the European Cystic Fibrosis Society Patient Registry.
Authors: Orenti A, Boeck K, Kerem E, Burgel PR, Zolin A, Bakkeheim E, Naehrlich L, Hatziagorou E; ECFSPR Steering Group Committee.Lancet Respir Med. 2026 Jun 3:S2213-2600(26)00149-9. doi: 10.1016/S2213-2600(26)00149-9. Online ahead of print.PMID: 42235564
ECFS statement on theratyping and theranostics in the context of rare and ultrarare CFTR variants in people with CF.
Authors: De Boeck K, Burgel PR, Bierlaagh M, Hill K, Amaral M, Birimberg-Schwartz L, Brewington JJ, Davies JC, Karadag B, Martin U, Mense M, Pedemonte N, Sharma N, Tayor-Cousar JL, Sermet I, Beekman JM.J Cyst Fibros. 2026 May 6:S1569-1993(26)00105-0. doi: 10.1016/j.jcf.2026.04.013. Online ahead of print.PMID: 42097909 Free article. Review.
Determinants of early Elexacaftor-Tezacaftor-Ivacaftor use in adults with cystic fibrosis and preserved lung function: insights from a European multicenter survey
Authors: Gramegna A, Magnoni C, Premuda C, Alicandro G, Akkerman O, Amorim A, Azevedo P, Bourke S, Carnovale V, Diamantea F, Fila L, Frost F, Giron Moreno RM, Pescaru C, Polverino E, Felipe Montiel A, Rachel M, Stoyanova GP, Van Braeckel E, Volpi S, Vukić-Dugac A, Wege S, Simmonds NJ, Plant BJ, Downey DG, van Koningsbruggen-Rietschel S, Burgel PR, Blasi F.J Cyst Fibros. 2026 Mar 19:S1569-1993(26)00050-0. doi: 10.1016/j.jcf.2026.03.005. Online ahead of print.PMID: 41862298 Free article.
The impact of progressive expansion of elexacaftor-tezacaftor-ivacaftor availability on lung transplant for cystic fibrosis in France.
Authors: Dhôte T, Grenet D, Reynaud-Gaubert M, Chatron E, Renaud-Picard B, Tissot A, Macey J, Murris-Espin M, Bunel V, Sermet-Gaudelus I, Carlier N, Boussaud V, Kanaan R, Legeai C, Dorent R, de Miranda S, Da Silva J, Burgel PR.J Cyst Fibros. 2026 Jul;25(4):664-669. doi: 10.1016/j.jcf.2026.03.003. Epub 2026 Mar 11.PMID: 41813505 Free article.
Evaluating Long-Term Effectiveness of Cystic Fibrosis Modulator Therapies after Rapid Adoption: A Dual-Approach Study.
Authors: Miranda Afonso P, Zhou GC, Su W, Burgel PR, Cromwell E, Goss CH, Keogh RH, Liou TG, Marshall BC, Mayer-Hamblett N, Morgan WJ, Ostrenga JS, Pasta DJ, Schechter MS, Stanojevic S, Wainwright CE, Szczesniak RD, Andrinopoulou ER.Ann Am Thorac Soc. 2026 Jan 1;23(1):89-99. doi: 10.1513/AnnalsATS.202503-267OC.PMID: 40920855 Free PMC article.
Reply: Lung organoids: a new frontier in neonatology and paediatric respiratory medicine.
Authors: Zanetto L, Bonadies L, Moll-Diaz R, Beekman J, Muraca M, Pozzobon M, Baraldi E.Eur Respir Rev. 2026 Mar 25;35(179):260009. doi: 10.1183/16000617.0009-2026. Print 2026 Jan.PMID: 41881456 Free PMC article.
Therapeutic hopes for CFTR-related recurrent pancreatitis: a case-series.
Authors: Gohy S, Le A, Sermet-Gaudelus I, De Wachter E, Lhomme A, Vermeulen F, Scheers I.J Cyst Fibros. 2026 May;25(3):509-512. doi: 10.1016/j.jcf.2026.02.008. Epub 2026 Feb 23.PMID: 41735157
Pediatric lung ultrasound: Tips and tricks for better scanning and interpretation.
Authors: Musolino AM, Gori L, Tei M, Supino MC, D’Amore C, Ciofi Degli Atti M, Di Sarno L, Amendolea A, Manganaro A, Soldati G, Tursi F, Buonsenso D, De Rose C, Cutrera R, Cirillo M, Tomà P, Bock C, Esposito F, Villani A, Colacino V, Iadecola A, Adamoli P, Trobia GL, Coco AD, Limoli G, Agostiniani R.Multidiscip Respir Med. 2026 Feb 20;21(1):1074. doi: 10.5826/mrm.2026.1074.PMID: 41718590 Free PMC article
Improvement in functional exercise performance and quadriceps muscle strength after two years treatment with elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis: A prospective observational study.
Authors: Aubriot AS, Song C, Gonçalvès C, Santelé L, Berardis S, Goubau C, Reychler G, Penelle M, Gohy S.J Cyst Fibros. 2026 Jul 20:S1569-1993(26)01657-7. doi: 10.1016/j.jcf.2026.07.003. Online ahead of print.PMID: 42476895
Feasibility of an innovative follow-up method alternating face-to-face consultations and teleconsultations for adult patients with cystic fibrosis (pwCF) treated with elexacaftor/tezacaftor/ivacaftor (ETI) for at least one year.
Authors: Thomas V, Isabelle D, Marie P, Rachel RD, Mael R, Yoann R, Quitterie R.Respir Med Res. 2026 May;89:101258. doi: 10.1016/j.resmer.2026.101258. Epub 2026 Mar 18.PMID: 41936292 Free article.
Transformations within cystic fibrosis model of care in the era of CFTR modulators: a Scoping Review.
Authors: Gautiez R, Reix P, Poupon-Bourdy S, Reynaud Q, Fayolle R, Cornec S, Durieu I, Haesebaert J.Respir Med. 2026 Jun 30;261:109014. doi: 10.1016/j.rmed.2026.109014. Online ahead of print.PMID: 42379530
2025
Monitoring ETI effects over 1.7 years in an infant treated in utero, via breast milk and granules by repeated faecal elastase measurements.
Authors: Appelt D.. J Cyst Fibros.. 05/2025. doi: 10.1016/j.jcf.2025.01.012.
The CARMUCI Study Design: A Double-Blind, Cross-Over Sham-Controlled Trial of Indoor Air Purification in People With Cystic Fibrosis and Primary Ciliary Dyskinesia.
Authors: Kakkoura M, et al. Pediatric Pulmonology. 02/2026. doi: doi: 10.1002/ppul.71514.
Real-world effectiveness and safety of elexacaftor/tezacaftor/ivacaftor in cystic fibrosis patients with Phe508del -gating and -residual function genotypes.
Authors: Salvatore D. Respir Med.. 01/2025. doi: 10.1016/j.rmed.2025.
Height Velocity in Pediatric Cystic Fibrosis Under Triple CFTR Modulator Therapy: A Real-Life Monocentric Experience.
Authors: Boni A. J Clin Med. 07/2025. doi: 10.3390/jcm14155259.
Pregnancies in women with cystic fibrosis in the era of CFTR modulators: Current challenges and future perspectives. Insights from the Italian cystic fibrosis registry.
Authors: Salvatore M. J Cyst Fibros. 12/2025. doi: 10.1016/j.jcf.2025.12.002.
ARIA-Italy multidisciplinary consensus on nasal polyposis and biological treatments: Update 2025.
Authors: Lombardi C. World Allergy Organ J. 05/2025. doi: 10.1016/j.waojou.2025.101058.
Immunoglobulin A mucosal immunity in people with cystic fibrosis shortly after initiation of highly effective modulator therapy.
Authors: Mottais A. ERJ Open Research. 11/2025. doi: 10.1183/23120541.01369-2024. PMID: 41220816
Sweat chloride and lung function responses to elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis with two versus one responsive CFTR variants: an analysis of two real-world observational studies.
Authors: Burgel PR. Lancet Respir Med. 11/2025. doi: 10.1016/S2213-2600(25)00190-0.
Pulmonary endpoints in clinical trials for children with cystic fibrosis under two years of age.
Authors: Lee T. J Cyst Fibros. 07/2025. doi: 10.1016/j.jcf.2025.06.003.
Sex-Specific Differences in Chronic Thromboembolic Pulmonary Hypertension Treated with Balloon Pulmonary Angioplasty.
Authors: Kurzyna Paweł. Journal of Clinical Medicine. 01/2025. doi: https://doi.org/10.3390/jcm14030899.
The Importance of Dose Escalation in the Treatment of Pulmonary Arterial Hypertension with Treprostinil.
Authors: Kędzierski Piotr. Biomedicines. 01/2025. doi: https://doi.org/10.3390/biomedicines13010172.
Analysis of demographic and clinical data of patients treated at the adult cystic fibrosis centre, department of pulmonology, hospital of lithuanian university of health sciences kauno klinikos, 2012–2023.
Authors: Kamilė Kaluževičė, Kęstutis Malakauskas, Virginija Kalinauskaitė-Zukauskė. Pulmonologija ir alergologija. 06/2025. doi: https://doi.org/10.37499/PIA.1702.
Cystic fibrosis treatment news.
Authors: Virginija kalinauskaitė-žukauskė. Pulmonologija ir alergologija. 06/2025. doi: https://doi.org/10.37499/PIA.1698.
Gene expression profile of intestinal organoids from people with cystic fibrosis upon exposure to elexacaftor/tezacaftor/ivacaftor.
Authors: Lucie Bořek-Dohalská, Tereza Doušová, Štěpánka Novotná, Eva Furstová, Ondřej Cinek, Klára Hubáčková, Pavel Dřevínek. Journal of Cystic Fibrosis. 01/2025. doi: 10.1016/j.jcf.2024.09.005. PMID: 39278758
Personalized Medicine in Cystic Fibrosis: Characterization of Eight Rare CFTR Variants in Intestinal Organoids and Cellular Models.
Authors: Tereza Doušová. Molecular Diagnosis & Therapy. 01/2025. doi: 10.1007/s40291-025-00806-5. PMID: 39278758 PMCID: PMC12847090
Vanzacaftor-tezacaftor-deutivacaftor versus elexacaftor-tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years and older (SKYLINE Trials VX20-121-102 and VX20-121-103): results from two randomised, active-controlled, phase 3 trials.
Authors: Pavel Dřevínek (collaborator). The Lancet (Respiratory Medicine). 03/2025. doi: 10.1016/S2213-2600(24)00411-9. PMID: 39756424 PMCID: PMC12184100
Beyond Trikafta: new models to assess tissue dependent rescue of N1303K-CFTR.
Authors: Pranke I. Front Pharamcol. 12/2025. doi: 10.3389/fphar.2025.1661417.
Adenine base editing with engineered virus-like particles rescues the CFTR mutation G542X in patient-derived intestinal organoids.
Authors: Nicosia L. iScience. 03/2025. doi: 10.1016/j.isci.2025.111979.
Effect of elexacaftor-tezacaftor-ivacaftor on bronchial dilatations in adolescents with cystic fibrosis: a multicentre prospective observational study.
Authors: Sermet-Gaudelus. Lancet Respiratory J. 12/2025. doi: 10.1016/S2213-2600(25)00248-. PMID: 41138737
Health inequity in people with cystic fibrosis: can we close the gap?
Authors: Egil Bakkeheim. Annals of the American Thoracic Society. 09/2025. doi: 10.1513/AnnalsATS.202501-052OC. PMID: 40929684
Elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis harbouring two CFTR Class I variants: real-world data from the French compassionate programme.
Authors: Burgel PR. EClinicalMedicine. 10/2025. doi: 10.1016/j.eclinm.2025.
Diffuse Cystic Lung Disease: A Clinical Guide to Recognition and Management.
Authors: Franciosi AN, Gupta N, Murphy DJ, Wikenheiser-Brokamp KA, McCarthy C. Chest. 167/2025. doi: 10.1016/j.chest.2024.08.008
CFTR Modulator Therapy Impacts Neutrophil CD39 Expression in Cystic Fibrosis.
Authors: Gogoi D, Gabillard-Lefort C, Baird R, Forde L, Niu M, Ahmed SW, Gunaratnam C, Williamson M, Casey M, Reeves EP. Am J Respir Cell Mol Biol. 2025. doi: 10.1165/rcmb.2025-0262LE.
Characterizing individuals with elevated sweat chloride results in the absence of CFTR variants.
Authors: Bhuiyan I, Chou FY, Roberts JM, Franciosi A, Quon BS. Orphanet J Rare Dis. 21/2025. doi: 10.1186/s13023-025-04145-w.
Reduction in 3-Year Risk of Death or Lung Transplant for Individuals with Advanced Cystic Fibrosis Lung Disease Treated with Elexacaftor/Tezacaftor/Ivacaftor.
Authors: Love AJ, Desai S, Cheng SY, Stephenson AL, Stanojevic S, Franciosi AN, Quon BS. Ann Am Thorac Soc. 22/2025. doi: 10.1513/AnnalsATS.202405-451RL.
Global prevalence of CFTR variants with respect to their responsiveness to elexacaftor-tezacaftor-ivacaftor.
Authors: Pierre-Régis Burgel et al, on the behalf of the ECFSPR scientific committee and the Global CF Registry Collaboration, 2 persons (Tiina Kahre and M.Vasar) from Estonia as Global CF collaboration. J Cyst Fibros. 11/2025. doi: 10.1016/j.jcf.2025.10.007. PMID: 41109837 PMCID: none / not specified.
Real-world experiences with interleukin-5 pathway inhibition in severe eosinophilic asthma: A European registry analysis.
Authors: Pelaia C. Allergy. 07/2025. doi: 10.1111/all.15237.
The novel CFAbd-Score.kidⒸ reveals a significant decline of abdominal symptoms in children with Cystic fibrosis aged 6 through 11 years on Elexacaftor/Tezacaftor/Ivacaftor – first results.
Authors: Eickmeier O.Journal of Cystic Fibrosis. 09/2025. doi: https://doi.org/10.1016/j.jcf.2025.09.003. PMID: 40946087
Validation of the CFAbd-Score.kidⒸ, a Novel gastrointestinal patient reported outcome measure, specific for children with cystic fibrosis.
Authors: Eickmeier O.Journal of Cystic Fibrosis. 09/2025. doi: https://doi.org/10.1016/j.jcf.2025.07.010. PMID: 40701924
Future treatment paradigms in pulmonary arterial hypertension: a personal view from physicians, health authorities, and patients. Authors: Rahaghi FF. Lancet Respir Med. 04/2025. doi: 10.1016/S2213-2600(24)00425-9.
MedBot vs RealDoc: efficacy of large language modeling in physician-patient communication for rare diseases.
Authors: Buda A. J Am Med Inform Assoc. 05/2025. doi: 10.1093/jamia/ocae087.
Lung organoids: a new frontier in neonatology and paediatric respiratory medicine.
Authors: Zanetto L. Eur Respir Rev. 08/2025. doi: 10.1183/16000617.0255-2024.
A Unique Combination of Heterozygous CFTR Gene Variants in a Person with Cystic Fibrosis and Mycobacterium abscessus Infection.
Authors: Lemson A. Respiration. 09/2025. doi: 10.1159/000545552.
Pulmonary arteriole narrowing in end-stage cystic fibrosis lungs occurs with and without small airway disease.
Authors: Wuyts W.A.. JOURNAL OF CYSTIC FIBROSIS. 09/2025. doi: 10.1016/j.jcf.2025.04.002.
2024
Improved Nutritional Outcomes and Gastrointestinal Symptoms in Adult Cystic Fibrosis Patients Treated with Elexacaftor/Tezacaftor/Ivacaftor
Authors: Stastna, N., Kunovsky, L., Svoboda, M., Pokojova, E., Homola, L., Mala, M., Gracova, Z., Jerabkova, B., Skrickova, J., & Trna, J. (2024). Digestive Diseases, 42(4), 361–368. https://doi.org/10.1159/000538606
Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues.
Burgel PR, Southern KW, Addy C, Battezzati A, Berry C, Bouchara JP, Brokaar E, Brown W, Azevedo P, Durieu I, Ekkelenkamp M, Finlayson F, Forton J, Gardecki J, Hodkova P, Hong G, Lowdon J, Madge S, Martin C, McKone E, Munck A, Ooi CY, Perrem L, Piper A, Prayle A, Ratjen F, Rosenfeld M, Sanders DB, Schwarz C, Taccetti G, Wainwright C, West NE, Wilschanski M, Bevan A, Castellani C, Drevinek P, Gartner S, Gramegna A, Lammertyn E, Landau EEC, Plant BJ, Smyth AR, van Koningsbruggen-Rietschel S, Middleton PG.J Cyst Fibros. 2024 Mar;23(2):187-202. doi: 10.1016/j.jcf.2024.01.005. Epub 2024 Jan 16.PMID: 38233247 Free article. Review.
Prime editing functionally corrects cystic fibrosis-causing CFTR mutations in human organoids and airway epithelial cells.
Bulcaen M, Kortleven P, Liu RB, Maule G, Dreano E, Kelly M, Ensinck MM, Thierie S, Smits M, Ciciani M, Hatton A, Chevalier B, Ramalho AS, Casadevall I Solvas X, Debyser Z, Vermeulen F, Gijsbers R, Sermet-Gaudelus I, Cereseto A, Carlon MS.Cell Rep Med. 2024 May 21;5(5):101544. doi: 10.1016/j.xcrm.2024.101544. Epub 2024 May 1.PMID: 38697102 Free PMC article.
Gathering real-world compassionate data to expand eligibility for elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis with N1303K or other rare CFTR variants: a viewpoint.
Burgel PR, Sermet-Gaudelus I, Girodon E, Kanaan R, Le Bihan J, Remus N, Ravoninjatovo B, Grenet D, Porzio M, Houdouin V, Le Clainche-Viala L, Durieu I, Nove-Josserand R, Languepin J, Coltey B, Guillaumot A, Audousset C, Chiron R, Weiss L, Fajac I, Da Silva J, Martin C; French CF Reference Network study group.Eur Respir J. 2024 Jan 25;63(1):2301959. doi: 10.1183/13993003.01959-2023. Print 2024 Jan.PMID: 38242629 No abstract available.
The expanded French compassionate programme for elexacaftor-tezacaftor-ivacaftor use in people with cystic fibrosis without a F508del CFTR variant: a real-world study.
Burgel PR, Sermet-Gaudelus I, Girodon E, Durieu I, Houdouin V, Audousset C, Macey J, Grenet D, Porzio M, Murris-Espin M, Reix P, Baravalle M, Belleguic C, Mely L, Verhille J, Weiss L, Reynaud-Gaubert M, Mittaine M, Hamidfar R, Ramel S, Cosson L, Douvry B, Danner-Boucher I, Foucaud P, Roy C, Burnet E, Raynal C, Audrezet MP, Da Silva J, Martin C; French Cystic Fibrosis Reference Network study group.Lancet Respir Med. 2024 Nov;12(11):888-900. doi: 10.1016/S2213-2600(24)00208-X. Epub 2024 Aug 13.PMID: 39151434
New drugs, new challenges in cystic fibrosis care.
Fajac I, Burgel PR, Martin C.Eur Respir Rev. 2024 Sep 25;33(173):240045. doi: 10.1183/16000617.0045-2024. Print 2024 Jul.PMID: 39322262 Free PMC article. Review.
Multisystemic Effects of Elexacaftor-Tezacaftor-Ivacaftor in Adults with Cystic Fibrosis and Advanced Lung Disease.
Burgel PR, Paillasseur JL, Durieu I, Reynaud-Gaubert M, Hamidfar R, Murris-Espin M, Danner-Boucher I, Chiron R, Leroy S, Douvry B, Grenet D, Mely L, Ramel S, Montcouquiol S, Burnet E, Ouaalaya EH, Sogni P, Da Silva J, Martin C; Participating Investigators of the French Cystic Fibrosis Reference Network Study Group.Ann Am Thorac Soc. 2024 Jul;21(7):1053-1064. doi: 10.1513/AnnalsATS.202312-1065OC.PMID: 38579175
Flemish network on rare connective tissue diseases (CTD): patient pathways in systemic sclerosis. First steps taken.
Piette Y, Van den Bossche F, Aerts J, Aerts N, Ajeganova S, Badot V, Berghen N, Blockmans D, Brusselle G, Caeyers N, De Decker M, De Haes P, De Cock C, De Keyser F, De Langhe E, Delcroix M, De Nutte H, De Pauw M, Depicker A, De Sutter A, De Sutter J, Du Four T, Frank C, Goubau J, Guiot J, Gutermuth J, Heeman L, Houssiau F, Hennes I, Lenaerts J, Lintermans A, Loeys B, Luyten H, Maeyaert B, Malfait F, Moeyersoons A, Mostmans Y, Nijs J, Poppe B, Polfliet K, Ruttens D, Sabato V, Schoeters E, Slabbynck H, Stuer A, Tamirou F, Thevissen K, Van Kersschaever G, Vanneuville B, Van Offel J, Vanthuyne M, Van Wabeke J, Verbist C, Vos I, Westhovens R, Wuyts W, Yserbyt J, Smith V.Acta Clin Belg. 2024 Feb;79(1):26-33. doi: 10.1080/17843286.2023.2280737. Epub 2024 Jan 2.PMID: 38108332 Free article
Lumacaftor/Ivacaftor Population Pharmacokinetics in Pediatric Patients with Cystic Fibrosis: A First Step Toward Personalized Therapy.
Bouazza N, Urien S, Foissac F, Choupeaux L, Lui G, Froelicher Bournaud L, Rouillon S, Zheng Y, Bardin E, Stremler N, Bessaci K, Bihouee T, Coirier-Duet E, Marguet C, Deneuville E, Laurans M, Reix P, Gerardin M, Mittaine M, Epaud R, Thumerelle C, Weiss L, Berthaud R, Semeraro M, Treluyer JM, Benaboud S, Sermet-Gaudelus I.Clin Pharmacokinet. 2024 Mar;63(3):333-342. doi: 10.1007/s40262-023-01342-3. Epub 2024 Feb 4.PMID: 38310629
PREFUL MRI for Monitoring Perfusion and Ventilation Changes after Elexacaftor-Tezacaftor-Ivacaftor Therapy for Cystic Fibrosis: A Feasibility Study.
Dohna M, Voskrebenzev A, Klimeš F, Kaireit TF, Glandorf J, Pallenberg ST, Ringshausen FC, Hansen G, Renz DM, Wacker F, Dittrich AM, Vogel-Claussen J.Radiol Cardiothorac Imaging. 2024 Apr;6(2):e230104. doi: 10.1148/ryct.230104.PMID: 38573129 Free PMC article.
Reversal of cylindrical bronchial dilatations in a subset of adults with cystic fibrosis treated with elexacaftor/tezacaftor/ivacaftor.
Cazier P, Chassagnon G, Dhote T, Da Silva J, Kanaan R, Honoré I, Carlier N, Revel MP, Canniff E, Martin C, Burgel PR.Eur Respir J. 2024 Mar 28;63(3):2301794. doi: 10.1183/13993003.01794-2023. Print 2024 Mar.PMID: 38331460
Monitoring of ECFS quality standards for the clinical management of adults with cystic fibrosis.
Gramegna A, Aliberti S, Amorim A, Blasi F, Bourke S, Burgel PR, Diamantea F, Durieu I, Fila L, Moreno RMG, Messore B, Pokojová E, Taccetti G, Verhulst S, Dugac AV, Wege S, Duff A, Southern KW, Castellani C.J Cyst Fibros. 2024 Mar;23(2):306-313. doi: 10.1016/j.jcf.2023.11.001. Epub 2023 Nov 8.PMID: 37949745
Kidney effects of triple CFTR modulator therapy in people with cystic fibrosis.
Gabai P, Novel-Catin E, Reynaud Q, Nove-Josserand R, Pelletier S, Fouque D, Koppe L, Durieu I.Clin Kidney J. 2024 Aug 27;17(10):sfae256. doi: 10.1093/ckj/sfae256. eCollection 2024 Oct.PMID: 39359568 Free PMC article.
Abnormal functional lymphoid tolerance and enhanced myeloid exocytosis are characteristics of resting and stimulated PBMCs in cystic fibrosis patients.
Gaudin C, Ghinnagow R, Lemaire F, Villeret B, Sermet-Gaudelus I, Sallenave JM.Front Immunol. 2024 Feb 26;15:1360716. doi: 10.3389/fimmu.2024.1360716. eCollection 2024.PMID: 38469306 Free PMC article.
Health care resource utilization preceding death or lung transplantation in people with cystic fibrosis: HCRU before transplant or death in cystic fibrosis.
Guyot E, Reynaud Q, Belhassen M, Bérard M, Dehillotte C, Lemonnier L, Viprey M, Van Ganse E, Burgel PR, Durieu I.J Cyst Fibros. 2024 Sep;23(5):903-909. doi: 10.1016/j.jcf.2024.03.001. Epub 2024 Mar 12.PMID: 38480112
Centralized intestinal organoid generation is a feasible and safe approach for personalized medicine as demonstrated in the HIT-CF Europe Organoid Study.
Bierlaagh MC, van Mourik P, Vonk AM, Pott J, Muilwijk D, Berkers G, Aalbers BL, Vleggaar FP, Michel S, Boj SF, Vries RGJ, Beekman JM, van der Ent CK; HIT-CF organoid study group.J Cyst Fibros. 2024 Jul;23(4):703-706. doi: 10.1016/j.jcf.2024.04.016. Epub 2024 May 18.PMID: 38763840 Free article.
Intimacy and sexual life of females with cystic fibrosis.
Simonnet Bisson F, Fauvernier M, Belleguic C, Boucher ID, Grenet D, Hamidfar R, Hubert D, Macey J, Murris-Espin M, Porzio M, Ramel S, Reynaud Q, Reynaud-Gaubert M, Burgel PR.J Cyst Fibros. 2024 Aug 24:S1569-1993(24)00829-4. doi: 10.1016/j.jcf.2024.08.003. Online ahead of print.PMID: 39183126
One year of ETI reduces lung bacterial colonisation in adults with cystic fibrosis.
Mianowski L, Doléans-Jordheim A, Barraud L, Rabilloud M, Richard M, Josserand RN, Durieu I, Reynaud Q.Sci Rep. 2024 Nov 26;14(1):29298. doi: 10.1038/s41598-024-77246-4.PMID: 39592637 Free PMC article.
Inhibiting CFTR through inh-172 in primary neutrophils reveals CFTR-specific functional defects.
Da Silva Cunha AL, Blanter M, Renders J, Gouwy M, Lorent N, Boon M, Struyf S, Carlon MS.Sci Rep. 2024 Dec 28;14(1):31237. doi: 10.1038/s41598-024-82535-z.PMID: 39732786 Free PMC article.
Ceftolozane/Tazobactam for the Treatment of Adults With Cystic Fibrosis: Results From a French Prospective Cohort Study.
Authors: Burgel PR, Bourge X, Mackosso C, Parquin F.Open Forum Infect Dis. 2024 Aug 6;11(8):ofae391. doi: 10.1093/ofid/ofae391. eCollection 2024 Aug.PMID: 39108933 Free PMC article.
The Challenging Life of Mutators: How Pseudomonas aeruginosa Survives between Persistence and Evolution in Cystic Fibrosis Lung. Microorganisms
Authors: Rossitto M, Fox V, Vrenna G, Tuccio Guarna Assanti V, Essa N, Lepanto MS, Raimondi S, Agosta M, Cortazzo V, Fini V, Granaglia A, Montemitro E, Cutrera R, Perno CF, Bernaschi P. 2024, T2024 Oct 11;12(10):2051. doi:10.3390/microorganisms12102051. PMID: 39458360; PMCID: PMC11509988.
Use of Lung Ultrasound in Cystic Fibrosis: Is It a Valuable Tool?
Authors: Boni A, Cristiani L, Majo F, Ullmann N, Esposito M, Supino MC, Tomà P, Villani A, Musolino AM, Cutrera R. 2024. Children (Basel). 2024 Jul 30;11(8):917. doi:10.3390/children11080917. PMID: 39201852; PMCID: PMC11352880.
Reported Adverse Events in a Multicenter Cohort of Patients Ages 6-18 Years with Cystic Fibrosis and at Least One F508del Allele Receiving Elexacaftor/Tezacaftor/Ivacaftor.
Authors: Terlizzi V, Fevola C, Presti S, Castaldo A, Daccò V, Claut L, Sepe A, Majo F, Casciaro R, Esposito I, Vitullo P, Salvi M, Troiani P, Ficili F, Parisi GF, Pantano S, Costa S, Leonetti G, Palladino N, Taccetti G, Bonomi P, Salvatore D. 2024. J Pediatr. 2024 Nov;274:114176. doi:10.1016/j.jpeds.2024.114176. Epub 2024 Jun 28. Erratum in: J Pediatr. 2024 Nov;274:114228. doi: 10.1016/j.jpeds.2024.114228. PMID: 38945446.
Gruppo di lavoro RIFC. Registro Italiano Fibrosi Cistica (RIFC). Rapporto 2021-2022 [Italian Cystic Fibrosis Registry (ICFR). Report 2021-2022].
Authors: Campagna G, Amato A, Majo F, Ferrari G, Quattrucci S, Padoan R, Floridia G, Salvatore D, Carnovale V, Puppo Fornaro G, Taruscio D, Salvatore M; 2024. Epidemiol Prev. 2024 Mar-Apr;48(2 Suppl 2):1-41. Italian. doi: 10.19191/EP24.2.S2.031. Erratum in: Epidemiol Prev. 2024 May-Jun;48(3):192. doi: 10.19191/EP24.3.ERRATA.053. PMID: 38742380.
Covid-19 in cystic fibrosis patients compared to the general population: Severity and virus-host cell interactions
Authors: Ciciriello F, Panariello F, Medino P, Biffi A, Alghisi F, Rosazza C, Annunziata P, Bouchè V, Grimaldi A, Guidone D, Venturini A, Alicandro G, Oggioni M, Cerino P, Paiola G, Gramegna A, Fiocchi A, Bandera A, Lucidi V, Cacchiarelli D, Galietta LJV, Colombo C.. J Cyst Fibros. 2024 Jul;23(4):625-632. doi: 10.1016/j.jcf.2024.03.006. Epub 2024 Mar 19. PMID: 38508950.
CFTR function is impaired in a subset of patients with pancreatitis carrying rare CFTR variants.
Authors: Angyal D, Kleinfelder K, Ciciriello F, Groeneweg TA, De Marchi G, de Pretis N, Bernardoni L, Rodella L, Tomba F, De Angelis P, Surace C, Pintani E, Alghisi F, de Jonge HR, Melotti P, Sorio C, Lucidi V, Bijvelds MJC, Frulloni L Pancreatology. 2024 May;24(3):394-403. doi: 10.1016/j.pan.2024.03.005. Epub 2024 Mar 10. PMID: 38493004.
Year in review 2023 – Back to the future
Authors: Cristiani L, Fernandes FF.. J Cyst Fibros. 2024 Mar;23(2):203-207. doi: 10.1016/j.jcf.2024.02.007. Epub 2024 Mar 2. PMID: 38431442.
Lung clearance index short-term variability in cystic fibrosis: a pre-post pulmonary exacerbation study
Authors: De Marchis M, Montemitro E, Boni A, Federici A, Di Giovanni D, Cristiani L, Cutrera R, Fiocchi AG.. Ital J Pediatr. 2024 Jan 17;50(1):6. doi: 10.1186/s13052-023-01574-w. PMID: 38233941; PMCID: PMC10795345.
Gene expression profile of intestinal organoids from people with cystic fibrosis upon exposure to elexacaftor/tezacaftor/ivacaftor
Authors: Cinek O, Furstova E, Novotna S, Hubackova K, Dousova T, Borek-Dohalska L, Drevinek P.. J Cyst Fibros. 2025 Jan;24(1):157-163. doi: 10.1016/j.jcf.2024.09.005. Epub 2024 Sep 14. PMID: 39278758 : Gene expression profile of intestinal organoids from people with cystic fibrosis upon exposure to elexacaftor/tezacaftor/ivacaftor – PubMed
Global CF Registry Collaboration. Impact of COVID-19 infection on lung function and nutritional status amongst individuals with cystic fibrosis: A global cohort study
Authors: Semenchuk J, Naito Y, Charman SC, Carr SB, Cheng SY, Marshall BC, Faro A, Elbert A, Gutierrez HH, Goss CH, Karadag B, Burgel PR, Colombo C, Salvatore M, Padoan R, Daneau G, Harutyunyan S, Kashirskaya N, Kirwan L, Middleton PG, Ruseckaite R, de Monestrol I, Naehrlich L, Mondejar-Lopez P, Jung A, van Rens J, Bakkeheim E, Orenti A, Zomer-van Ommen D, da Silva-Filho LVR, Fernandes FF, Zampoli M, Stephenson AL;. J Cyst Fibros. 2024 Sep;23(5):815-822. doi: 10.1016/j.jcf.2024.07.019. Epub 2024 Aug 26. PMID: 39191560: Impact of COVID-19 infection on lung function and nutritional status amongst individuals with cystic fibrosis: A global cohort study – PubMed
Standards for the care of people with cystic fibrosis (CF); Planning for a longer life.
Authors: Gramegna A, Addy C, Allen L, Bakkeheim E, Brown C, Daniels T, Davies G, Davies JC, De Marie K, Downey D, Felton I, Hafkemeyer S, Hamouda S, Kendall V, Lindberg U, Macek M, Mayell S, Pearlsman O, Schechter MS, Salvatori L, Sands D, Schwarz C, Shteinberg M, Taylor J, Taylor-Cousar JL, Taylor-Robinson D, Watkins B, Verkleij M, Bevan A, Castellani C, Drevinek P, Gartner S, Lammertyn E, Landau EEC, Middleton PG, Plant BJ, Smyth AR, van Koningsbruggen-Rietschel S, Burgel PR, Southern KW. J Cyst Fibros. 2024 May;23(3):375-387. doi: 10.1016/j.jcf.2024.05.007. Epub 2024 May 24. PMID: 38789317: Standards for the care of people with cystic fibrosis (CF); Planning for a longer life – PubMed
Precision medicine in cystic fibrosis: predictive role of forskolin-induced swelling assay.
Authors: Furstova E, Drevinek P, Novotna S, Libik M, Benesova K, Borek-Dohalska L, Sakmarova K, Modrak M, Macek M Jr, Dousova T. Eur Respir J. 2024 Apr 18;63(4):2400156. doi: 10.1183/13993003.00156-2024. PMID: 38485147: Precision medicine in cystic fibrosis: predictive role of forskolin-induced swelling assay – PubMed
Standards for the care of people with cystic fibrosis; establishing and maintaining health.
Authors: Southern KW, Addy C, Bell SC, Bevan A, Borawska U, Brown C, Burgel PR, Button B, Castellani C, Chansard A, Chilvers MA, Davies G, Davies JC, De Boeck K, Declercq D, Doumit M, Drevinek P, Fajac I, Gartner S, Georgiopoulos AM, Gursli S, Gramegna A, Hansen CM, Hug MJ, Lammertyn E, Landau EEC, Langley R, Mayer-Hamblett N, Middleton A, Middleton PG, Mielus M, Morrison L, Munck A, Plant B, Ploeger M, Bertrand DP, Pressler T, Quon BS, Radtke T, Saynor ZL, Shufer I, Smyth AR, Smith C, van Koningsbruggen-Rietschel S. J Cyst Fibros. 2024 Jan;23(1):12-28. doi: 10.1016/j.jcf.2023.12.002. Epub 2023 Dec 21. PMID: 38129255: Standards for the care of people with cystic fibrosis; establishing and maintaining health – PubMed
Ivacaftor pharmacokinetics and lymphatic transport after enteral administration in rats.
Authors: Pozniak J, Ryšánek P, Smrčka D, Kozlík P, Křížek T, Šmardová J, Nováková A, Das D, Bobek D, Arora M, Hofmann J, Doušová T, Šíma M, Slanař O. Front Pharmacol. 2024 Feb 20;15:1331637.doi: 10.3389/fphar.2024.1331637. PMID: 38444938; PMCID: PMC10912587: Ivacaftor pharmacokinetics and lymphatic transport after enteral administration in rats – PubMed
Impaired lung function in infants with primary ciliary dyskinesia: A pilot Czech study.
Authors: Koucký V, Martinů V, Koucký M. Pediatr Pulmonol. 2024 Apr;59(4):1124-1127. doi: 10.1002/ppul.26863. Epub 2024 Jan 11. PMID: 38206044 : Impaired lung function in infants with primary ciliary dyskinesia: A pilot Czech study – PubMed
Letter to the editor: Risk of false newborn screening after intra-uterine exposure to ETI.
Authors: De Wachter E, Davies JC, Simmonds NJ, Castellani C, de Winter-de Groot KM, Munck A, Proesmans M, Southern KW, Barben J. J Cyst Fibros. 2024 Jan;23(1):176-177. doi: 10.1016/j.jcf.2023.07.003. Epub 2023 Jul 24. PMID: 37495469.
ECFS Diagnostic Network Working Group. ECFS standards of care on CFTR-related disorders: Identification and care of the disorders.
Authors: Simmonds NJ, Southern KW, De Wachter E, De Boeck K, Bodewes F, Mainz JG, Middleton PG, Schwarz C, Vloeberghs V, Wilschanski M, Bourrat E, Chalmers JD, Ooi CY, Debray D, Downey DG, Eschenhagen P, Girodon E, Hickman G, Koitschev A, Nazareth D, Nick JA, Peckham D, VanDevanter D, Raynal C, Scheers I, Waller MD, Sermet-Gaudelus I, Castellani C; J Cyst Fibros. 2024 Jul;23(4):590-602. doi: 10.1016/j.jcf.2024.03.008. Epub 2024 Mar 19. PMID: 38508949.
ECFS Diagnostic Network Working Group. ECFS standards of care on CFTR-related disorders: Towards a comprehensive program for affected individuals
Authors: De Wachter E, De Boeck K, Sermet-Gaudelus I, Simmonds NJ, Munck A, Naehrlich L, Barben J, Boyd C, Veen SJ, Carr SB, Fajac I, Farrell PM, Girodon E, Gonska T, Grody WW, Jain M, Jung A, Kerem E, Raraigh KS, van Koningsbruggen-Rietschel S, Waller MD, Southern KW, Castellani C;. J Cyst Fibros. 2024 May;23(3):388-397. doi: 10.1016/j.jcf.2024.01.012. Epub 2024 Feb 22. PMID: 38388234.
Impact of the expanded label for elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis with no F508del variant in the United States.
Authors: Cromwell EA, Ostrenga JS, Sanders DB, Morgan W, Castellani C, Szczesniak R, Burgel PR. Eur Respir J 2024; in press
Expanding the indication of CFTR modulator combinations for people with cystic fibrosis with non-F508del variants.
Authors: Burgel PR. Lancet Respir Med 2024; 12(12):934-935
Cystic Fibrosis.
Authors: Mall M, Burgel PR, Castellani C, Davies J, Salathe M, Taylor-Cousar J. Nat Rev Dis Primers 2024; 10:53.
Standards for the care of people with cystic fibrosis; Planning for a longer life.
Authors: Gramegna A, Addy C, Allen L, Bakkeheim E, Brown C, Daniels T, Davies G , Davies JC , De Marie K, Downey D, Felton I, Hafkemeyer S, Hamouda S, Kendall V, Lindberg U, Macek M, Mayell S, Pearlsman O, Schechter MS, Salvatori L, Sands D, Schwarz C, Shteinberg M, Taylor J, Taylor-Cousar JL, Taylor-Robinson D, Watkins B, Verkleij M, Bevan A, Castellani C, Drevinek P, Gartner S, Lammertyn E, Landau EC, Middleton PG, Plant BJ, Smyth AR, van Koningsbruggen-Rietschel S, Burgel PR, Kevin W Southern KW. J Cyst Fibros 2024; 23(3):375-387.
Considerations for the use of inhaled antibiotics for Pseudomonas aeruginosa in people with cystic fibrosis receiving CFTR modulator therapy.
Authors: Burgel PR, Ballmann M, Drevinek P, Heijerman H, Jung A, Mainz JG, Peckham D, Plant BJ, Schwarz C, Taccetti G, Smyth A BMJ Open Respir Res 2024; 11(1):e002049.
“Supporting the case for a targeted approach for elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis with no F508del CFTR variant – further analysis for the French Compassionate programme”.
Authors: Burgel PR. Reply to Eur Respir J 2024; 63(2):2400233.
Cystic fibrosis in the era of CFTR modulators: did the neutrophil slip through the cracks ?
Authors: Witko-Sarsat V, Burgel PR. J Leukoc Biol 2024; 115(3):417-419.
Dual Role of microRNA-146a in Experimental Inflammation in Human Pulmonary Epithelial and Immune Cells and Expression in Inflammatory Lung Diseases.
Authors: Gronau L, Duecker RP, Jerkic SP, Eickmeier O, Trischler J, Chiocchetti AG, Blumchen K, Zielen S, Schubert R. Int J Mol Sci. 2024 Jul 13;25(14):7686. doi: 10.3390/ijms25147686.PMID: 39062931 Free PMC article.
Proinflammatory pattern in the lower airways of non-asthmatic obese adolescents.
Authors: Gutmann D, Dressler M, Eickmeier O, Herrmann E, Kirwil M, Schubert R, Zielen S, Zissler UM. Cytokine. 2024 Jan;173:156452. doi: 10.1016/j.cyto.2023.156452. Epub 2023 Nov 30.PMID: 38039695
Cystic fibrosis patient characteristics and healthcare resource utilization in Finland using linked registries.
Authors: Kirsi Malmivaara, Mari Pölkki, Tuire Prami, Paavo Raittinen, Eija Heikkilä, Antti Aalto, Teija Dunder, Varpu Elenius, Kristina Sandström, Lisa J McGarry. Heliyon. 2024 Jun 22;10(13):e33439. doi: 10.1016/j.heliyon.2024. e33439. eCollection 2024 Jul 15.
Mitigating the risk of inflammatory type primary graft dysfunction by applying an integrated approach to assess, modify and match risk factors in lung transplantation.
Authors: Braithwaite SA, Berg EM, de Heer LM, Jennekens J, Neyrinck A, van Hooijdonk E, Luijk B, Buhre WFFA, van der Kaaij NP. Front Transplant. 2024 Aug 20;3:1422088. doi: 10.3389/frtra.2024.1422088. eCollection 2024.PMID: 39229386
Organoid-guided synergistic treatment of minimal function CFTR mutations with CFTR modulators, roflumilast and simvastatin: a personalised approach.
Authors: Spelier S, de Winter-de Groot K, Keijzer-Nieuwenhuijze N, Liem Y, van der Ent K, Beekman J, Kamphuis LS. Eur Respir J. 2024 Jan 25;63(1):2300770. doi: 10.1183/13993003.00770-2023. Print 2024 Jan. PMID: 37857424
BMI increase during early childhood in boys with cystic fibrosis and early adrenarche
Authors: Gizem Tamer 1 2, Hubertus Gerardus Maria Arets 2, Cornelis Kors van der Ent 2, Hanneke Margo van Santen 1 3, Hetty Jacoba van der Kamp 1 Pediatr Pulmonol. 2024 Apr;59(4):991-996. doi: 10.1002/ppul.26861. Epub 2024 Jan 25
European guidelines for the diagnosis and treatment of pancreatic exocrine insufficiency: UEG, EPC, EDS, ESPEN, ESPGHAN, ESDO, and ESPCG evidence-based recommendations.
Authors: Dominguez-Muñoz JE, Vujasinovic M, de la Iglesia D, Cahen D, Capurso G, Gubergrits N, Hegyi P, Hungin P, Ockenga J, Paiella S, Perkhofer L, Rebours V, Rosendahl J, Salvia R, Scheers I, Szentesi A, Bonovas S, Piovani D, Löhr JM; European PEI Multidisciplinary Group.United European Gastroenterol J. 2025
The intermittent intrapulmonary deflation technique for airway clearance in patients with cystic fibrosis: A randomized trial.
Authors: de Macedo JRFF, Aubriot AS, Reychler G, Penelle M, Gohy S, Poncin W.Respir Med Res. 2024
One year effect of tezacaftor and ivacaftor on functional exercise capacity and muscle strength in people with cystic fibrosis.
Authors: Anne-Sophie A, Penelle M, Clémence G, Berardis S, Goubau C, Reychler G, Gohy S.Heliyon. 2024
Novel time-saving OGTT sparing HbA1c-HOMA2 based algorithm for the diagnosis of cystic fibrosis-related diabetes.
Authors: Lurquin F, Gohy S, Hermans MP, Preumont V.Diabetes Res Clin Pract. 2024
European guidelines for the diagnosis and treatment of pancreatic exocrine insufficiency: UEG, EPC, EDS, ESPEN, ESPGHAN, ESDO, and ESPCG evidence-based recommendations.
Authors: Dominguez-Muñoz JE, Vujasinovic M, de la Iglesia D, Cahen D, Capurso G, Gubergrits N, Hegyi P, Hungin P, Ockenga J, Paiella S, Perkhofer L, Rebours V, Rosendahl J, Salvia R, Scheers I, Szentesi A, Bonovas S, Piovani D, Löhr JM; European PEI Multidisciplinary Group.United European Gastroenterol J. 2025
The intermittent intrapulmonary deflation technique for airway clearance in patients with cystic fibrosis: A randomized trial.
Authors: de Macedo JRFF, Aubriot AS, Reychler G, Penelle M, Gohy S, Poncin W.Respir Med Res. 2024
One year effect of tezacaftor and ivacaftor on functional exercise capacity and muscle strength in people with cystic fibrosis.
Authors: Anne-Sophie A, Penelle M, Clémence G, Berardis S, Goubau C, Reychler G, Gohy S.Heliyon. 2024
Novel time-saving OGTT sparing HbA1c-HOMA2 based algorithm for the diagnosis of cystic fibrosis-related diabetes.
Authors: Lurquin F, Gohy S, Hermans MP, Preumont V.Diabetes Res Clin Pract. 2024
ESPEN-ESPGHAN-ECFS guideline on nutrition care for cystic fibrosis.
Authors: Wilschanski M, Munck A, Carrion E, Cipolli M, Collins S, Colombo C, Declercq D, Hatziagorou E, Hulst J, Kalnins D, Katsagoni CN, Mainz JG, Ribes-Koninckx C, Smith C, Smith T, Van Biervliet S, Chourdakis M. Clin Nutr. 2024
2023
The French Compassionate Program of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis with advanced lung disease and no F508del CFTR variant.
Authors: Burgel PR, Sermet-Gaudelus I, Durieu I, Kanaan R, Macey J, Grenet D, Porzio M, Coolen-Allou N, Chiron R, Marguet C, Douvry B, Dufeu N, Danner-Boucher I, Foucaud P, Lemonnier L, Girodon E, Da Silva J, Martin C; French CF Reference Network study group.Eur Respir J. 2023 Feb 16:2202437. doi: 10.1183/13993003.02437-2022. Online ahead of print.PMID: 36796836
Cystic fibrosis.
Authors: Fajac I, Burgel PR.Presse Med. 2023 Jul 27;52(3):104169. doi: 10.1016/j.lpm.2023.104169. Online ahead of print.PMID: 37516246
Lung transplantation for end-stage cystic fibrosis before and after the availability of elexacaftor-tezacaftor-ivacaftor, Germany, 2012-2021.
Authors: Ringshausen FC, Sauer-Heilborn A, Büttner T, Dittrich AM, Schwerk N, Ius F, Nährlich L, Welte T, Greer M.Eur Respir J. 2023 Jan 12;61(1):2201402. doi: 10.1183/13993003.01402-2022. Print 2023 Jan.PMID: 36517178 No abstract available.
Lung Function Decline in Cystic Fibrosis: Impact of Data Availability and Modeling Strategies on Clinical Interpretations.
Authors: Szczesniak R, Andrinopoulou ER, Su W, Afonso PM, Burgel PR, Cromwell E, Gecili E, Ghulam E, Goss CH, Mayer-Hamblett N, Keogh RH, Liou TG, Marshall B, Morgan WJ, Ostrenga JS, Pasta DJ, Stanojevic S, Wainwright C, Zhou GC, Fernandez G, Fink AK, Schechter MS.Ann Am Thorac Soc. 2023 Jul;20(7):958-968. doi: 10.1513/AnnalsATS.202209-829OC.PMID: 36884219
Bronchiectasis in Europe: data on disease characteristics from the European Bronchiectasis registry (EMBARC).
Authors: Chalmers JD, Polverino E, Crichton ML, Ringshausen FC, De Soyza A, Vendrell M, Burgel PR, Haworth CS, Loebinger MR, Dimakou K, Murris M, Wilson R, Hill AT, Menendez R, Torres A, Welte T, Blasi F, Altenburg J, Shteinberg M, Boersma W, Elborn JS, Goeminne PC, Aliberti S; EMBARC Registry Investigators.Lancet Respir Med. 2023 Jul;11(7):637-649. doi: 10.1016/S2213-2600(23)00093-0. Epub 2023 Apr 24.PMID: 37105206 Free article.
Theratyping cystic fibrosis patients to guide elexacaftor/tezacaftor/ivacaftor out-of-label prescription.
Authors: Dreano E, Burgel PR, Hatton A, Bouazza N, Chevalier B, Macey J, Leroy S, Durieu I, Weiss L, Grenet D, Stremler N, Ohlmann C, Reix P, Porzio M, Roux Claude P, Rémus N, Douvry B, Montcouquiol S, Cosson L, Mankikian J, Languepin J, Houdouin V, Le Clainche L, Guillaumot A, Pouradier D, Tissot A, Priou P, Mély L, Chedevergne F, Lebourgeois M, Lebihan J, Martin C, Zavala F, Da Silva J, Lemonnier L, Kelly-Aubert M, Golec A, Foucaud P, Marguet C, Edelman A, Hinzpeter A, de Carli P, Girodon E, Sermet-Gaudelus I, Pranke I; French CF Reference Network study group.Eur Respir J. 2023 Oct 19;62(4):2300110. doi: 10.1183/13993003.00110-2023. Print 2023 Oct.PMID: 37696564
Cystic fibrosis and the cardiovascular system: the unexpected heartache.
Authors: Hebestreit H, Thornton CS.Eur Respir J. 2023 Oct 26;62(4):2301253. doi: 10.1183/13993003.01253-2023. Print 2023 Oct.PMID: 37884293 No abstract available.
The Changing Epidemiology of Cystic Fibrosis: The Implications for Adult Care.
Authors: Burgel PR, Burnet E, Regard L, Martin C.Chest. 2023 Jan;163(1):89-99. doi: 10.1016/j.chest.2022.07.004. Epub 2022 Jul 16.PMID: 35850286 Review.
Transforming clinical research and science in bronchiectasis: EMBARC3, a European Respiratory Society Clinical Research Collaboration.
Authors: Chalmers JD, Aliberti S, Altenburg J, Blasi F, Clarke C, Chotirmall SH, Crichton ML, Dhar R, Goeminne P, Haworth C, Loebinger MR, Lorent N, Polverino E, Ringshausen FC, Shoemark A, Shteinberg M, Sibila O, Spinou A, Welte T.Eur Respir J. 2023 Jun 29;61(6):2300769. doi: 10.1183/13993003.00769-2023. Print 2023 Jun.PMID: 37385653 No abstract available.
Long-term safety and efficacy of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis and at least one F508del allele: 144-week interim results from a 192-week open-label extension study.
Authors: Daines CL, Tullis E, Costa S, Linnemann RW, Mall MA, McKone EF, Polineni D, Quon BS, Ringshausen FC, Rowe SM, Selvadurai H, Taylor-Cousar JL, Withers NJ, Ahluwalia N, Moskowitz SM, Prieto-Centurion V, Tan YV, Tian S, Weinstock T, Xuan F, Zhang Y, Ramsey B, Griese M; VX17-445-105 Study Group.Eur Respir J. 2023 Dec 7;62(6):2202029. doi: 10.1183/13993003.02029-2022. Print 2023 Dec.PMID: 37945033 Free PMC article. Clinical Trial.
Validating organoid-derived human intestinal monolayers for personalized therapy in cystic fibrosis.
Authors: Birimberg-Schwartz L, Ip W, Bartlett C, Avolio J, Vonk AM, Gunawardena T, Du K, Esmaeili M, Beekman JM, Rommens J, Strug L, Bear CE, Moraes TJ, Gonska T.Life Sci Alliance. 2023 Apr 5;6(6):e202201857. doi: 10.26508/lsa.202201857. Print 2023 Jun.PMID: 37024122 Free PMC article.
Exploring intrinsic variability between cultured nasal and bronchial epithelia in cystic fibrosis.
Authors: Rodenburg LW, Metzemaekers M, van der Windt IS, Smits SMA, den Hertog-Oosterhoff LA, Kruisselbrink E, Brunsveld JE, Michel S, de Winter-de Groot KM, van der Ent CK, Stadhouders R, Beekman JM, Amatngalim GD.Sci Rep. 2023 Oct 30;13(1):18573. doi: 10.1038/s41598-023-45201-4.PMID: 37903789 Free PMC article.
French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis – 2021 update. Full-length version.
Authors: Cottin V, Bonniaud P, Cadranel J, Crestani B, Jouneau S, Marchand-Adam S, Nunes H, Wémeau-Stervinou L, Bergot E, Blanchard E, Borie R, Bourdin A, Chenivesse C, Clément A, Gomez E, Gondouin A, Hirschi S, Lebargy F, Marquette CH, Montani D, Prévot G, Quetant S, Reynaud-Gaubert M, Salaun M, Sanchez O, Trumbic B, Berkani K, Brillet PY, Campana M, Chalabreysse L, Chatté G, Debieuvre D, Ferretti G, Fourrier JM, Just N, Kambouchner M, Legrand B, Le Guillou F, Lhuillier JP, Mehdaoui A, Naccache JM, Paganon C, Rémy-Jardin M, Si-Mohamed S, Terrioux P; OrphaLung network.Respir Med Res. 2023 Jun;83:100948. doi: 10.1016/j.resmer.2022.100948. Epub 2022 Aug 4.PMID: 36630775 Review.
Qualitative and quantitative evaluation of computed tomography changes in adults with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor: a retrospective observational study.
Authors: Dettmer S, Weinheimer O, Sauer-Heilborn A, Lammers O, Wielpütz MO, Fuge J, Welte T, Wacker F, Ringshausen FC.Front Pharmacol. 2023 Sep 21;14:1245885. doi: 10.3389/fphar.2023.1245885. eCollection 2023.PMID: 37808186 Free PMC article.
Protocol for generating airway organoids from 2D air liquid interface-differentiated nasal epithelia for use in a functional CFTR assay.
Authors: Rodenburg LW, van der Windt IS, Dreyer HHM, Smits SMA, den Hertog-Oosterhoff LA, Aarts EM, Beekman JM, Amatngalim GD.STAR Protoc. 2023 Sep 15;4(3):102337. doi: 10.1016/j.xpro.2023.102337. Epub 2023 Jun 12.PMID: 37314920 Free PMC article.
CFTR Modulators: Current Status and Evolving Knowledge.
Authors: Regard L, Martin C, Da Silva J, Burgel PR.Semin Respir Crit Care Med. 2023 Apr;44(2):186-195. doi: 10.1055/s-0042-1758851. Epub 2022 Dec 19.PMID: 36535667 Free article.
Monitoring of ECFS quality standards for the clinical management of adults with cystic fibrosis.
Authors: Gramegna A, Aliberti S, Amorim A, Blasi F, Bourke S, Burgel PR, Diamantea F, Durieu I, Fila L, Moreno RMG, Messore B, Pokojová E, Taccetti G, Verhulst S, Dugac AV, Wege S, Duff A, Southern KW, Castellani C.J Cyst Fibros. 2023 Nov 8:S1569-1993(23)01672-7. doi: 10.1016/j.jcf.2023.11.001. Online ahead of print.PMID: 37949745
Epidemiological trends in nontuberculous mycobacterial infection among people with cystic fibrosis in Germany.
Authors: Hafkemeyer S, Ruckes C, Stehling F, Naehrlich L, Ringshausen FC.Int J Infect Dis. 2023 Apr;129:32-39. doi: 10.1016/j.ijid.2023.01.032. Epub 2023 Jan 31.PMID: 36736578 Free article.
Proteomic profiling of sweat in patients with cystic fibrosis provides new insights into epidermal homoeostasis.
Authors: Cornet M, Nguyen-Khoa T, Kelly-Aubert M, Jung V, Chedevergne F, Le Bourgeois M, Aoust L, Roger K, Guerrera CI, Sermet-Gaudelus I.Skin Health Dis. 2022 Nov 25;3(1):e161. doi: 10.1002/ski2.161. eCollection 2023 Feb.PMID: 36751320 Free PMC article.
Normalisation of circulating neutrophil counts after 12 months of elexacaftor-tezacaftor-ivacaftor in patients with advanced cystic fibrosis.
Authors: Dhote T, Martin C, Regard L, Pesenti L, Kanaan R, Carlier N, Honoré I, Da Silva J, Witko-Sarsat V, Burgel PR.Eur Respir J. 2023 Jan 6;61(1):2202096. doi: 10.1183/13993003.02096-2022. Print 2023 Jan.PMID: 36455960 No abstract available.
CT imaging shows specific pancreatic abnormalities in persons with cystic fibrosis related diabetes.
Authors: Alexandre-Heymann L, Puerto M, Martin C, Burnet E, Mosnier-Pudar H, Burgel PR, Larger E.Sci Rep. 2023 Jun 27;13(1):10433. doi: 10.1038/s41598-023-37492-4.PMID: 37369827 Free PMC article.
Pseudomonas aeruginosa population genomics among adults with bronchiectasis across Germany.
Authors: Rosenboom I, Oguz S, Lüdemann IM, Ringshausen FC, Rademacher J, Sedlacek L, Tümmler B, Cramer N.ERJ Open Res. 2023 Jun 26;9(3):00156-2023. doi: 10.1183/23120541.00156-2023. eCollection 2023 May.PMID: 37377651 Free PMC article.
Outcome of lung transplantation for adults with interstitial lung disease associated with genetic disorders of the surfactant system.
Authors: Bermudez J, Nathan N, Coiffard B, Roux A, Hirschi S, Degot T, Bunel V, Le Pavec J, Macey J, Le Borgne A, Legendre M, Cottin V, Thomas PA, Borie R, Reynaud-Gaubert M.ERJ Open Res. 2023 Nov 20;9(6):00240-2023. doi: 10.1183/23120541.00240-2023. eCollection 2023 Nov.PMID: 38020562 Free PMC article.
The RISE study protocol: resilience impacted by positive stressful events for people with cystic fibrosis.
Authors: van der Heijden E, van den Bor RM, van der Ent CK, Nijhof SL, van der Laan SEI.ERJ Open Res. 2023 Jun 12;9(3):00535-2022. doi: 10.1183/23120541.00535-2022. eCollection 2023 May.PMID: 37313395 Free PMC article.
Optimal treatment of the underlying aetiology is the most effective antimicrobial stewardship for chronic respiratory disease: a lesson learned from cystic fibrosis.
Authors: Rademacher J, Martin L, Theloe A, Stahl M, Mall MA, Joean O, Fuge J, Hansen G, Welte T, Schütz K, Ringshausen FC, Dittrich AM.ERJ Open Res. 2023 Aug 29;9(4):00356-2023. doi: 10.1183/23120541.00356-2023. eCollection 2023 Jul.PMID: 37650087 Free PMC article.
Spirometric and anthropometric improvements in response to elexacaftor/tezacaftor/ivacaftor depending on age and lung disease severity.
Authors: Schütz K, Pallenberg ST, Kontsendorn J, DeLuca D, Sukdolak C, Minso R, Büttner T, Wetzke M, Dopfer C, Sauer-Heilborn A, Ringshausen FC, Junge S, Tümmler B, Hansen G, Dittrich AM.Front Pharmacol. 2023 Jul 4;14:1171544. doi: 10.3389/fphar.2023.1171544. eCollection 2023.PMID: 37469865 Free PMC article.
The U UGA C sequence provides a favorable context to ELX-02 induced CFTR readthrough.
Authors: Pranke IM, Varilh J, Hatton A, Faucon C, Girodon E, Dreano E, Chevalier B, Karri S, Reix P, Durieu I, Bidou L, Namy O, Taulan M, Hinzpeter A, Sermet-Gaudelus I.J Cyst Fibros. 2023 May;22(3):560-563. doi: 10.1016/j.jcf.2022.10.010. Epub 2022 Nov 16.PMID: 36400713 No abstract available.
Elexacaftor-Tezacaftor-Ivacaftor treatment reduces abdominal symptoms in cystic fibrosis-early results obtained with the CF-specific CFAbd-Score.
Authors: Eickmeier O, Smaczny C. Front Pharmacol. 2023 May 3;14:1207356.
Transforming Growth Factor ß1 and Gap Junction Protein Alpha 4 Gene Heterogeneity in Relation to the Severity of Clinical Disease in Cystic Fibrosis.
Authors: Eickmeier O, Smaczny C. Front Biosci (Landmark Ed). 2023 Jul 19;28(7):138.
Impact of elexacaftor/tezacaftor/ivacaftor on lung function, nutritional status, pulmonary exacerbation frequency and sweat chloride in people with cystic fibrosis: real-world evidence from the German CF Registry.
Authors: Smaczny C, Eickmeier O, German CF Registry of the Mukoviszidose e.V. and participating CF sites.]Lancet Reg Health Eur. 2023 Jul 28;32:100690.
Dynamics of abdominal symptoms during the start of a new therapy with elexacaftor/tezacaftor/ivacaftor using the novel CFAbd-day2day questionnaire.
Authors: Eickmeier O, Van Dullemen S Front Pharmacol. 2023 Oct 25;14:1167407.
Nationwide lung function monitoring from infancy in newborn-screened children with cystic fibrosis.
Authors: Sandvik RM, Schmidt MN, Voldby CM, Buchvald FF, Olesen HV, Olsen J, Kragh MV, Rubak SLM, Pressler T, Robinson PD,
Gustafsson PM, Skov M, Nielsen KG.
ERJ Open Res. 2023 Oct 30;9(5):00317-2023. doi: 10.1183/23120541.00317-2023. eCollection 2023 Sep.
PMID: 37908398 Free PMC article.
Automatic analysis of bronchus-artery dimensions to diagnose and monitor airways disease in cystic fibrosis.
Authors: Lv Q, Gallardo-Estrella L, Andrinopoulou ER, Chen Y, Charbonnier JP, Sandvik RM, Caudri D, Nielsen KG, de Bruijne M, Ciet P,
Tiddens H. Thorax. 2023 Dec 15;79(1):13-22. doi: 10.1136/thorax-2023-220021.
PMID: 37734952 Free PMC article.
Antimicrobial resistance of Pseudomonas aeruginosa in a cystic fibrosis population after introduction of a novel cephalosporin/βlactamase inhibitor
Authors: Katzenstein TL, Faurholt-Jepsen D, Qvist T, Jensen PØ, Pressler T, Johansen HK, Kolpen M.
APMIS. 2023 Aug;131(8):419-425. doi: 10.1111/apm.13331. Epub 2023 Jun 9. PMID: 3729491110.
Outcome of elexacaftor/tezacaftor/ivacaftor therapy in patients with cystic fibrosis and solid organ transplantation.
Authors: Ørum MB, Rönsholt FF, Jeppesen M, Bendstrup E, Katzenstein TL, Ott P, Perch M, Pressler T, Qvist T, Jensen-Fangel S. Pediatr Pulmonol. 2023 Feb;58(2):602-605. doi: 10.1002/ppul.26217. Epub 2022 Nov 11.
PMID: 36314653
Characterization of impaired beta and alpha cell function in response to an oral glucose challenge in cystic fibrosis: a cross-sectional
study.
Authors: Nielsen BU, Mathiesen IHM, Møller R, Krogh-Madsen R, Katzenstein TL, Pressler T, Shaw JAM, Ritz C, Rickels MR, Stefanovski D,
Almdal TP, Faurholt-Jepsen D. Front Endocrinol (Lausanne). 2023 Aug 31;14:1249876. doi: 10.3389/fendo.2023.1249876. eCollection 2023.PMID: 37720541 Free PMC article.
Changes in glucose tolerance in people with cystic fibrosis after initiation of first-generation CFTR modulator treatment.
Authors: Ekblond RS, Nielsen BU, Højte C, Almdal TP, Shaw J, Pressler T, Faurholt-Jepsen D, Mathiesen IHM. Pediatr Pulmonol. 2023Sep;58(9):2600-2609. doi: 10.1002/ppul.26562. Epub 2023 Jun 21.
PMID: 37341613
Selection and monitoring of ECFS Quality Standards for the clinical management of adults with cystic fibrosis.
Authors: Gramegna A, Aliberti S, Amorim A, Blasi F, Bourke S, Burgel PR, Diamantea F, Durieu I, Fila L, Girón Morenok RM, Messore B, Pokojovám E, Taccetti G, Verhulst S, Dugac AV, Wege S, Duff A, Southern KW, Castellani C. J Cyst Fibros 2023; in press
Nonsense mutations accelerate lung disease and decrease survival of Cystic Fibrosis children.
Authors: Orenti A, Pranke I, Faucon C, Varilh J, Hatton A, Golec A, Dehillotte C, Durieu I, Reix P, Burgel PR, Grenet D, Tasset C, Gachelin E, Perisson C, Lepissier A, Dreano E, Tondelier D, Chevalier B, Weiss L, Kiefer S, Laurans M, Chiron R, Lemonnier L, Marguet C, Jung A, Edelman A, Kerem B, Girodon E, Taulan-Cadars M, Hinzpeter A, Kerem E, Naehrlich L, Sermet-Gaudelus I, on behalf of ECFSPR Steering group**. J Cyst Fibros 2023; 22(6):1070-1079.
Cystic Fibrosis Cases Missed by Newborn Bloodspot Screening-Towards a Consistent Definition and Data Acquisition.
Authors: Munck A, Southern KW, Murphy J, de Winter-de Groot KM, Gartner S, Karadag B, Kashirskaya N, Linnane B, Proesmans M, Sands D, Sommerburg O, Castellani C, Barben J; European CF Society Neonatal Screening Working Group (ECFS NSWG). Int J Neonatal Screen. 2023 Nov 21;9(4):65. doi: 10.3390/ijns9040065. PMID: 38132824; PMCID: PMC10743499.
Dynamic regulation of airway surface liquid pH by TMEM16A and SLC26A4 in cystic fibrosis nasal epithelia with rare mutations.
Authors: Delpiano L, Rodenburg LW, Burke M, Nelson G, Amatngalim GD, Beekman JM, Gray MA. Proc Natl Acad Sci U S A. 2023 Nov 21;120(47):e2307551120. doi: 10.1073/pnas.2307551120. Epub 2023 Nov 15. PMID: 37967223; PMCID: PMC10666107.
Exploring intrinsic variability between cultured nasal and bronchial epithelia in cystic fibrosis.
Authors: Rodenburg LW, Metzemaekers M, van der Windt IS, Smits SMA, den Hertog- Oosterhoff LA, Kruisselbrink E, Brunsveld JE, Michel S, de Winter-de Groot KM, van der Ent CK, Stadhouders R, Beekman JM, Amatngalim GD. Sci Rep. 2023 Oct 30;13(1):18573. doi: 10.1038/s41598-023-45201-4. PMID: 37903789; PMCID: PMC10616285.
CFTR Function Restoration upon Elexacaftor/Tezacaftor/Ivacaftor Treatment in Patient-Derived Intestinal Organoids with Rare <i>CFTR</i> Genotypes. Authors: Lefferts JW, Bierlaagh MC, Kroes S, Nieuwenhuijze NDA, Sonneveld van Kooten HN, Niemöller PJ, Verburg TF, Janssens HM, Muilwijk D, van Beuningen SFB, van der Ent CK, Beekman JM. Int J Mol Sci. 2023 Sep 26;24(19):14539. doi: 10.3390/ijms241914539. PMID: 37833986; PMCID: PMC10572896.
Optimization of performance of Dutch newborn screening for cystic fibrosis.
Authors: Bouva MJ, Dankert-Roelse JE, van der Ploeg C, Verschoof-Puite RK, Zomer-van Ommen DD, Gille J, Jakobs BS, Heijnen M, de Winter-de Groot KM. J Cyst Fibros. 2023 Sep 14:S1569-1993(23)00905-0. doi: 10.1016/j.jcf.2023.09.001. Epub ahead of print. PMID: 37716879.
Development and validation of a novel personalized electronic patient-reported outcome measure to assess quality of life (Q-LIFE): a prospective observational study in people with Cystic Fibrosis.
Authors: Muilwijk D, van Paridon TJ, van der Heijden DC, Faber-Bisschop BM, Zomer-van Ommen DD, Heijerman HGM, van der Ent CK. EClinicalMedicine. 2023 Jul 27;62:102116. doi: 10.1016/j.eclinm.2023.102116. PMID: 37554124; PMCID: PMC10404867.
Letter to the editor: Risk of false newborn screening after intra-uterine exposure to ETI.
Authors: De Wachter E, Davies JC, Simmonds NJ, Castellani C, de Winter-de Groot KM, Munck A, Proesmans M, Southern KW, Barben J. J Cyst Fibros. 2023 Jul 24:S1569-1993(23)00832-9. doi: 10.1016/j.jcf.2023.07.003. Epub ahead of print. PMID: 37495469.
Protocol for generating airway organoids from 2D air liquid interface-differentiated nasal epithelia for use in a functional CFTR assay.
Authors: Rodenburg LW, van der Windt IS, Dreyer HHM, Smits SMA, den Hertog-Oosterhoff LA, Aarts EM, Beekman JM, Amatngalim GD. STAR Protoc. 2023 Sep 15;4(3):102337. doi: 10.1016/j.xpro.2023.102337. Epub 2023 Jun 12. PMID: 37314920; PMCID: PMC10511861.
The RISE study protocol: resilience impacted by positive stressful events for people with cystic fibrosis.
Authors: van der Heijden E, van den Bor RM, van der Ent CK, Nijhof SL, van der Laan SEI. ERJ Open Res. 2023 Jun 12;9(3):00535-2022. doi: 10.1183/23120541.00535-2022. PMID: 37313395; PMCID: PMC10258717.
Radiological and long-term clinical response to elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis with advanced lung disease.
Authors: Aalbers BL, Mohamed Hoesein FAA, Hofland RW, Bronsveld I, Kruijswijk MA, Schotman S, Slingerland CW, Panhuis H, van der Ent CK, Heijerman HGM. Pediatr Pulmonol. 2023 Aug;58(8):2317-2322. doi: 10.1002/ppul.26486. Epub 2023 May 24. PMID: 37222401.
16S rRNA-Based Microbiota Profiling Assists Conventional Culture Analysis of Airway Samples from Pediatric Cystic Fibrosis Patients.
Authors: Kristensen M, de Koff EM, Chu ML, Groendijk S, Tramper-Stranders GA, de Winter-de Groot KM, Janssens HM, Tiddens HA, van Westreenen M, Sanders EAM, Arets BHGM, van der Ent CK, Prevaes SMPJ, Bogaert D. Microbiol Spectr. 2023 Jun 15;11(3):e0405722. doi: 10.1128/spectrum.04057-22. Epub 2023 May 18. PMID: 37199622; PMCID: PMC10269535.
FDA-approved drug screening in patient-derived organoids demonstrates potential of drug repurposing for rare cystic fibrosis genotypes.
Authors: de Poel E, Spelier S, Hagemeijer MC, van Mourik P, Suen SWF, Vonk AM, Brunsveld JE, Ithakisiou GN, Kruisselbrink E, Oppelaar H, Berkers G, de Winter de Groot KM, Heida-Michel S, Jans SR, van Panhuis H, Bakker M, van der Meer R, Roukema J, Dompeling E, Weersink EJM, Koppelman GH, Blaazer AR, Muijlwijk-Koezen JE, van der Ent CK, Beekman JM. J Cyst Fibros. 2023 May;22(3):548-559. doi: 10.1016/j.jcf.2023.03.004. Epub 2023 May 3. PMID: 37147251.
Defining and Measuring Resilience in Children with a Chronic Disease: a Scoping Review.
Authors: van der Laan SEI, Berkelbach van der Sprenkel EE, Lenters VC, Finkenauer C, van der Ent CK, Nijhof SL. Advers Resil Sci. 2023;4(2):105-123. doi: 10.1007/s42844-023-00092-2. Epub 2023 Apr 10. PMID: 37139096; PMCID: PMC10088629.
Targeted locus amplification reveals heterogeneity between and within CFTR genotypes and association with CFTR function in patient-derived intestinal organoids.
Authors: Lefferts JW, Boersma V, Nieuwenhuijze NDA, Suen SWF, Hajo K, Collantes NS, Vermeulen C, Groeneweg T, Hagemeijer MC, de Jonge HR, van der Ent CK, Splinter E, Beekman JM. J Cyst Fibros. 2023 May;22(3):538-547. doi: 10.1016/j.jcf.2023.04.003. Epub 2023 Apr 24. PMID: 37100706.
Validating organoid-derived human intestinal monolayers for personalized therapy in cystic fibrosis.
Authors: Birimberg-Schwartz L, Ip W, Bartlett C, Avolio J, Vonk AM, Gunawardena T, Du K, Esmaeili M, Beekman JM, Rommens J, Strug L, Bear CE, Moraes TJ, Gonska T. Life Sci Alliance. 2023 Apr 5;6(6):e202201857. doi: 10.26508/lsa.202201857. PMID: 37024122; PMCID: PMC10079552.
Readthrough compounds for nonsense mutations: bridging the translational gap.
Authors: Spelier S, van Doorn EPM, van der Ent CK, Beekman JM, Koppens MAJ. Trends Mol Med. 2023 Apr;29(4):297-314. doi:10.1016/j.molmed.2023.01.004. Epub 2023 Feb 22. PMID: 36828712.
High- throughput functional assay in cystic fibrosis patient-derived organoids allows drug repurposing.
Authors: Spelier S, de Poel E, Ithakisiou GN, Suen SWF, Hagemeijer MC, Muilwijk D, Vonk AM, Brunsveld JE, Kruisselbrink E, van der Ent CK, Beekman JM. ERJ Open Res. 2023 Jan 30;9(1):00495-2022. doi: 10.1183/23120541.00495-2022. PMID: 36726369; PMCID: PMC9885274.
Use of 2,6-diaminopurine as a potent suppressor of UGA premature stop codons in cystic fibrosis.
Authors: Leroy C, Spelier S, Essonghe NC, Poix V, Kong R, Gizzi P, Bourban C, Amand S, Bailly C, Guilbert R, Hannebique D, Persoons P, Arhant G, Prévotat A, Reix P, Hubert D, Gérardin M, Chamaillard M, Prevarskaya N, Rebuffat S, Shapovalov G, Beekman J, Lejeune F. Mol Ther. 2023 Apr 5;31(4):970-985. doi: 10.1016/j.ymthe.2023.01.014. Epub 2023 Jan 14. PMID: 36641622; PMCID: PMC10124085.
Neonatal Screening Working Group (ECFS NSWG). European survey of newborn bloodspot screening for CF: opportunity to address challenges and improve performance.
Authors: Munck A, Berger DO, Southern KW, Carducci C, de Winter-de Groot KM, Gartner S, Kashirskaya N, Linnane B, Proesmans M, Sands D, Sommerburg O, Castellani C, Barben J; European CF Society J Cyst Fibros. 2023 May;22(3):484-495. doi: 10.1016/j.jcf.2022.09.012. Epub 2022 Nov 10. PMID: 36372700.
Dutch CF Registry Steering group. Epidemiology and management of nontuberculous mycobacterial disease in people with cystic fibrosis, the Netherlands.
Authors: Zomer D, van Ingen J, Hofland R; J Cyst Fibros. 2023 Mar;22(2):327-333. doi: 10.1016/j.jcf.2022.10.009. Epub 2022 Nov 6. PMID: 36347785.
22: Lefferts JW, Boersma V, Hagemeijer MC, Hajo K, Beekman JM, Splinter E. Targeted Locus Amplification and Haplotyping. Methods Mol Biol. 2023;2590:31-48. doi: 10.1007/978-1-0716-2819-5_2. PMID: 36335490.
Is Measuring Physical Literacy in School-Aged Children With Cystic Fibrosis or Congenital Heart Disease Needed?
Authors: Noordstar JJ, Sprong MCA, Slieker MG, Takken T, van Brussel M, van der Ent CK, Hulzebos EHJ. Pediatr Phys Ther. 2023 Jan 1;35(1):43-47. doi:10.1097/PEP.0000000000000967. Epub 2022 Sep 12. PMID: 36103630.
Development and outcomes of a patient driven cystic fibrosis quality of care improvement project.
Authors: Noordhoek JJ, Jeyaratnam J, Zomer D, Gulmans VAM, van der Ent CK, Heijerman HGM. J Cyst Fibros. 2023 Jan;22(1):172-178. doi: 10.1016/j.jcf.2022.07.004. Epub 2022 Jul 13. PMID: 35842291.
VOCAL: An observational study of ivacaftor for people with cystic fibrosis and selected non-G551D-CFTR gating mutations.
Authors: Simmonds NJ, van der Ent CK, Colombo C, Kinnman N, DeSouza C, Thorat T, Chew ML, Chandarana K, Castellani C. J Cyst Fibros. 2023 Jan;22(1):124-131. doi: 10.1016/j.jcf.2022.05.007. Epub 2022 May 23. PMID: 35613999.
Non-respiratory health-related quality of life in people with cystic fibrosis receiving elexacaftor/tezacaftor/ivacaftor.
Authors: Fajac, Isabelle ; Daines, Cori ; Durieu, Isabelle et al. / In: Journal of Cystic Fibrosis. 2023 ; Vol. 22, No. 1. pp. 119-123.
The prevalence of vascular and metabolic complications after lung transplant in people with cystic fibrosis in a Dutch cohort.
Authors: Oppelaar, Lisa M ; Luijk, Bart ; Heijerman, Harry G M et al. / In: Clinics (São Paulo, Brazil). 2023 ; Vol.78.
Improved Survival of IPF patients Treated With Antifibrotic Drugs Compared With Untreated Patients.
Authors: Platenburg, Mark G.J.P. ; van Moorsel, Coline H.M. ; Wiertz, Ivo A. et al. / In: Lung. 2023 ; Vol. 201, No. 4. pp. 335-343.
Thermal threshold testing : call for a balance between the number of measurements and abnormalities in the diagnosis of sarcoidosis-associated small fiber neuropathy
Authors: Raasing, Lisette R M ; Veltkamp, Marcel ; Datema, Mirjam et al. / . In: Pain reports. 2023 ; Vol. 8, No. 5.
Fully Automatic, Semiautomatic, and Manual Corneal Nerve Fiber Analysis in Patients With Sarcoidosis.
Authors: Raasing, Lisette R M ; Vogels, Oscar J M ; Datema, Mirjam et al. / In: Translational vision science & technology. 2023 ; Vol. 12, No. 12.
Feasibility and first results of the ‘Trials-within-Cohorts’ (TwiCs) design in patients undergoing radiotherapy for lung cancer.
Authors: Tomassen, Mathijs L; Damen, Pim J J ; Verkooijen, Helena M et al. / In: Acta Oncologica. 2023 ; Vol. 62, No. 3. pp. 237-244.
Epidemiology and management of nontuberculous mycobacterial disease in people with cystic fibrosis, the Netherlands.
Authors: Dutch CF Registry Steering group. In: Journal of Cystic Fibrosis. 2023 ; Vol. 22, No. 2. pp. 327-333.
Cystic Fibrosis: a real-world challenge to predict individual outcomes. Utrecht: Utrecht University, 2023. Thesis – Doctoral thesis 1 (Research UU / Graduation UU) Muilwijk, Danya. / 253 p.
A year in review (2022): Modulators and COVID19, the story goes on…
Authors: Dillenhoefer S, Grogono D, Morales-Tirado A. J Cyst Fibros. 202 Mar;22(2):188-192. doi: 10.1016/j.jcf.2023.03.001 . Epub 2023 Mar 6. PMID: 36906393 ; PMCID: PMC9986130.
Standards for the care of people with cystic fibrosis; establishing and maintaining health.
Authors: Southern KW, Addy C, Bell S, Bevan A, Borawska U, Brown C, Burgel PR, Button B, Castellani C, Chansard A,
Chilvers M, Davies G, Davies J, De Boeck K, Declercq D, Doumit M, Drevinek P, Fajac I, Gartner S, Georgiopoulos A, Gursli S, Gramegna A, Hansen CM, Hug M, Lammertyn E, Landau EEC, Langley R, Mayer Hamblett N, Middleton A, Middleton P, Mielus M, Morrison L, Munck A, Plant B, Ploeger M, Bertrand DP, Pressler T, Quon BS, Radtke T, Saynor ZL, Shufer I, Smyth AR, Smith C, West NE, van Koningsbruggen-Rietschel S. J Cyst Fibros. 2023 Dec 20:S1569-1993(23)01727-7
Cystic fibrosis transmembrane conductance regulator in COPD: a role in respiratory epithelium and beyond
Authors: Mall MA, Criner GJ, Miravitlles M, Rowe SM, Vogelmeier CF, Rowlands DJ, Schoenberger M, Altman P. Eur Respir J. 2023;61(4):2201307. FI; 24,9.
Standards for the care of people with cystic fibrosis (CF): A timely and accurate diagnosis.
Authors: Castellani C, Simmonds NJ, Barben J, Addy C, Bevan A, Burgel PR, Drevinek P, Gartner S, Gramegna A, Lammertyn E, Landau EEC, Middleton PG, Plant BJ, Smyth AR, van Koningsbruggen-Rietschel S, Girodon E, Kashirskaya N, Munck A, Nährlich L, Raraigh K, Sermet-Gaudelus I, Sommerburg O, Southern KW. J Cyst Fibros. 2023 Nov;22(6):963-968.
Omicron Wave Modification of the Coronavirus Disease 2019 Paradigm in Children With Cystic Fibrosis.
Authors: Thouvenin, G ; Prevost, B ; Corvol. J Infect Dis 35717636
Modifiers of Cystic Fibrosis Lung Disease Severity: Whole-Genome Analysis of 7,840 Patients.
Authors: Zhou, YH ; Gallins, PJ ; Pace, RG ; Dang, H ; Aksit, MA ; Blue, EE & al. Am J Respir Crit Care Med 36813289
Airway infections as a risk factor for Pseudomonas aeruginosa acquisition and chronic colonisation in children with cystic fibrosis.
Authors: Mésinèle, J ; Ruffin, M ; Guillot, L ; Boëlle, PY ; Corvol, H. J Cyst Fibros 37401873
Letter to the editor: Risk of false newborn screening after intra-uterine exposure to ETI.
Authors: De Wachter E, Davies JC, Simmonds NJ, Castellani C, de Winter-de Groot KM, Munck A, Proesmans M, Southern KW, Barben J. J Cyst Fibros. 2023 Jul 24:S1569-1993(23)00832-9. doi: 10.1016/j.jcf.2023.07.003. Epub ahead of print. PMID: 37495469
Chapter in book: Hodson and Geddes’ Cystic Fibrosis, 5th Edition, 2023. Chapter 17: Diagnostic Tests: Sweat Testing, Epithelial Potential Differences and Genetic Testing
Authors: Elke De Wachter, Caroline Raynal doi.org/10.1201/9781003262763 ISBN: 9781003262763
Risk factors for chronic colonization with Aspergillus fumigatus in people with cystic fibrosis October
2023Journal of Cystic Fibrosis 22:S18
Management of respiratory tract exacerbations in people with cystic fibrosis: Focus on imaging.
Authors: Landini N, Ciet P, Janssens HM, Bertolo S, Ros M, Mattone M, Catalano C, Majo F, Costa S, Gramegna A,
Lucca F, Parisi GF, Saba L, Tiddens HAWM, Morana G. Front Pediatr. 2023 Feb 6; 10:1084313. -Sellers ZM, Assis DN, Paranjape SM, Sathe M, Bodewes F, Bowen M, Cipolli M, Debray D, Green N, Hughan KS, Hunt WR, Leey J, Ling SC, Morelli G, Peckham D, Pettit RS, Philbrick A, Stoll J, Vavrina K, Allen S, Goodwin T, Hempstead SE, Narkewicz MR. Cystic fibrosis screening, evaluation and management of hepatobiliary disease consensus recommendations. Hepatology. 2023 Oct 26.
Traits, trends and hits of orphan drug designations in cystic fibrosis.
Authors: Costa E, Girotti S, van den Ham HA, Cipolli M, van der Ent CK, Taylor-Cousar JL, Leufkens HGM. J Cyst Fibros. 2023 Sep;22(5):949-957.
Clinical outcomes associated with Achromobacter species infection in people with cystic fibrosis.
Authors: Kerem E, Orenti A, Zolin A, Annicchiarico L, Drevinek P; ECFSPR with the list of contributing authors. J Cyst Fibros. 2023 Mar;22(2):334-343. IF 5,2
Elexacaftor-tezacaftor-ivacaftor in patients with cystic fibrosis ineligible for clinical trials: a 24-week observational study.
Authors: Fila L, Grandcourtova A, Bilkova A, Drevinek P. Front Pharmacol. 2023 Jun 2;14:1178009 IF 5,6
Comparison of amikacin lung delivery between AKITA® and eFlow rapid® nebulizers in healthy controls and patients with CF: A randomized cross-over trial.
Authors: Aubriot AS, Maerckx G, Leal T, Gohy S, Reychler G. Respir Med Res. 2023 Nov;84:101038
Combined CFTR modulator therapies are linked with anabolic benefits and insulin-sparing in cystic fibrosis-related diabetes.
Authors: Lurquin F, Gohy S, Hermans MP, Preumont V. J Clin Transl Endocrinol. 2023 Jun 24;33:100320
2022
ECFS standards of care on CFTR-related disorders: Diagnostic criteria of CFTR dysfunction.
Authors: Sermet-Gaudelus I, Girodon E, Vermeulen F, Solomon GM, Melotti P, Graeber SY, Bronsveld I, Rowe SM, Wilschanski M, Tümmler B, Cutting GR, Gonska T. J Cyst Fibros. 2022 Nov;21(6):922-936. doi: 10.1016/j.jcf.2022.09.005. Epub 2022 Oct 5. PMID: 36207272
Authors: Benoît Chevalier, Nesrine Baatallah, Matthieu Najm, Solène Castanier, Vincent Jung, Iwona Pranke, Anita Golec, Véronique Stoven, Stefano Marullo, Fabrice Antigny, Ida Chiara Guerrera, Isabelle Sermet-Gaudelus, Aleksander Edelman, Alexandre Hinzpeter. Int J Mol Sci. 2022 Aug 11;23(16):8937. doi: 10.3390/ijms23168937.
ECFS standards of care on CFTR-related disorders: Updated diagnostic criteria.
Authors: Castellani C, De Boeck K, De Wachter E, Sermet-Gaudelus I, Simmonds NJ, Southern KW; ECFS Diagnostic Network Working Group. J Cyst Fibros. 2022 Nov;21(6):908-921. doi: 10.1016/j.jcf.2022.09.011. Epub 2022 Oct 8. PMID: 36220763.
Authors: Rodenburg LW, Delpiano L, Railean V, Centeio R, Pinto MC, Smits SMA, van der Windt IS, van Hugten CFJ, van Beuningen SFB, Rodenburg RNP, van der Ent CK, Amaral MD, Kunzelmann K, Gray MA, Beekman JM, Amatngalim GD.Int J Mol Sci. 2022 Oct 21;23(20):12657. doi: 10.3390/ijms232012657.PMID: 36293514 Free PMC article.
The U UGA C sequence provides a favorable context to ELX-02 induced CFTR read through.
Authors: Iwona M Pranke, Jessica Varilh, Aurélie Hatton, Caroline Faucon, Emmanuelle Girodon, Elise Dreano, Benoit Chevalier, Sabrina Karri, Philippe Reix, Isabelle Durieu, Laure Bidou, Olivier Namy, Magali Taulan, Alexandre Hinzpeter, Isabelle Sermet-Gaudelus.
Tobramycin safety and efficacy review article.
Authors: Carsten Schwarz, Giovanni Taccetti, Pierre-Regis Burgel, Siobhain Mulrennan. Respir Med. 2022 Apr-May;195:106778.doi:10.1016/j.rmed.2022.106778. Epub 2022 Feb 25. PMID: 35249787DOI: 1016/j.rmed.2022.106778
Authors: Martin C, Reynaud-Gaubert M, Hamidfar R, Durieu I, Murris-Espin M, Danner-Boucher I, Chiron R, Leroy S, Douvry B, Grenet D, Mely L, Ramel S, Montcouquiol S, Lemonnier L, Burnet E, Paillasseur JL, Da Silva J, Burgel PR.J Cyst Fibros. 2022 May;21(3):489-496. doi: 10.1016/j.jcf.2022.01.012. Epub 2022 Feb 2.PMID: 35123901
Authors: Agathe Lepissier, Charlotte Addy, Kate Hayes, Sabrina Noel, Stéphanie Bui, Pierre-Régis Burgel, Lieven Dupont, Olaf Eickmeier, Michael Fayon, Teresinha Leal, Carlos Lopes, Damian G Downey, Isabelle Sermet-Gaudelus. J Cyst Fibros. 2022 Jul;21(4):691-706. doi: 10.1016/j.jcf.2021.10.009. Epub 2021 Nov 10.
Authors: Saha K, Chevalier B, Doly S, Baatallah N, Guilbert T, Pranke I, Scott MGH, Enslen H, Guerrera C, Chuon C, Edelman A, Sermet-Gaudelus I, Hinzpeter A, Marullo S.Cell Mol Life Sci. 2022 Sep 27;79(10):530. doi: 10.1007/s00018-022-04554-1.PMID: 36167862
CFTR Modulators in People with Cystic Fibrosis: Real-World Evidence in France.
Authors: Lucile Regard, Clémence Martin, Espérie Burnet, Jennifer Da Silva, Pierre-Régis Burgel. Cells. 2022 May 28;11(11):1769.doi: 10.3390/cells11111769.
Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice.
Authors: Mélanie Faria da Cunha, Iwona Pranke, Ali Sassi, Christiane Schreiweis, Stéphanie Moriceau, Dragana Vidovic, Aurélie Hatton, Mariane Sylvia Carlon, Geordie Creste, Farouk Berhal, Guillaume Prestat, Romain Freund, Norbert Odolczyk, Jean Philippe Jais, Christine Gravier-Pelletier, Piotr Zielenkiewicz, Vincent Jullien, Alexandre Hinzpeter, Franck Oury, Aleksander Edelman, Isabelle Sermet-Gaudelus. Sci Rep. 2022 Apr 12;12(1):6132.doi: 10.1038/s41598-022-09678-9
Authors: Gimano D Amatngalim, Lisa W Rodenburg, Bente L Aalbers, Henriette Hm Raeven, Ellen M Aarts, Dounia Sarhane, Sacha Spelier, Juliet W Lefferts, Iris Al Silva, Wilco Nijenhuis, Sacha Vrendenbarg, Evelien Kruisselbrink, Jesse E Brunsveld, Cornelis M van Drunen, Sabine Michel, Karin M de Winter-de Groot, Harry G Heijerman, Lukas C Kapitein, Magarida D Amaral, Cornelis K van der Ent, Jeffrey M Beekman. Life Sci Alliance. 2022 Aug 3;5(12):e202101320. doi: 10.26508/lsa.202101320. Print 2022 Dec.
The Changing Epidemiology of Cystic Fibrosis: The Implications for Adult Care.
Authors: Burgel PR, Burnet E, Regard L, Martin C. Chest. 2022 Jul 16:S0012-3692(22)01242-9. doi: 10.1016/j.chest.2022.07.004. Online ahead of print.PMID: 35850286 Review.
Major Decrease in Lung Transplantation for Patients with Cystic Fibrosis in France.
Authors: Martin C, Legeai C, Regard L, Cantrelle C, Dorent R, Carlier N, Kerbaul F, Burgel PR.Am J Respir Crit Care Med. 2022 Mar 1;205(5):584-586. doi: 10.1164/rccm.202109-2121LE.PMID: 34910604
Authors: Chassagnon G, Campredon A, Vakalopoulou M, Burgel PR.Eur Respir J. 2022 Jan 27:2200022. doi: 10.1183/13993003.00022-2022. Online ahead of print.PMID: 35086838
Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice.
Authors: da Cunha MF, Pranke I, Sassi A, Schreiweis C, Moriceau S, Vidovic D, Hatton A, Carlon MS, Creste G, Berhal F, Prestat G, Freund R, Odolczyk N, Jais JP, Gravier-Pelletier C, Zielenkiewicz P, Jullien V, Hinzpeter A, Oury F, Edelman A, Sermet-Gaudelus I.Sci Rep. 2022 Apr 12;12(1):6132. doi: 10.1038/s41598-022-09678-9.PMID: 35413967
Authors: Coriati A, Sykes J, Lemonnier L, Ma X, Stanojevic S, Dehillotte C, Carlier N, Stephenson AL, Burgel PR.Eur Respir J. 2022 Jan 27;59(1):2100014. doi: 10.1183/13993003.00014-2021. Print 2022 Jan.PMID: 34140297
Authors: Reix P, Tatopoulos A, Ioan I, Le Bourgeois M, Bui S, Choukroun ML, Bessaci-Kabouya K, Gerardin M, Bokov P, Da Silva J, Paillasseur JL, Burgel PR; French Cystic Fibrosis Reference Network study group.J Cyst Fibros. 2022 Jan;21(1):155-159. doi: 10.1016/j.jcf.2021.06.002. Epub 2021 Jun 26.PMID: 34183285
Authors: Veldhoen ES, Wijngaarde CA, Hulzebos EHJ, Wösten-van Asperen RM, Wadman RI, van Eijk RPA, Asselman FL, Stam M, Otto LAM, Cuppen I, Scheijmans FEV, den Oudenrijn LPV, Bartels B, Gaytant MA, van der Ent CK, van der Pol WL.Orphanet J Rare Dis. 2022 Feb 21;17(1):70. doi: 10.1186/s13023-022-02227-7.PMID: 35189949
Cumulative Incidence and Risk Factors for Severe COVID-19 in French People with Cystic Fibrosis.
Authors: Corvol H, de Miranda S, Dehillotte C, Lemonnier L, Chiron R, Danner-Boucher I, Hamidfar R, Houdouin V, Macey J, Marguet C, Murris-Espin M, Reynaud Q, Reix P, Gaubert MR, Kemgang A, Burgel PR; French Cystic Fibrosis Reference Network study group.Clin Infect Dis. 2022 Apr 27:ciac333. doi: 10.1093/cid/ciac333. Online ahead of print.PMID: 35475917
2021
Authors: Maximilian Zeyda, Andrea Schanzer, Pavel Basek, Vera Bauer, Ernst Eber, Helmut Ellemunter, Margit Kallinger, Josef Riedler, Christina Thir, Franz Wadlegger, Angela Zacharasiewicz, Sabine Renner. Diagnostics (Basel). 2021 Feb 13;11(2):299. doi: 10.3390/diagnostics11020299.
Authors: Sabine E I van der Laan, Marieke L A de Hoog, Sanne L Nijhof, Ulrike Gehring, Judith M Vonk, Cornelis K van der Ent, Alet H Wijga). J Pediatr. 2021 Jun;233:198-205.e2.doi: 10.1016/j.jpeds.2021.01.074. Epub 2021 Feb 4.
Authors: Diana Bilton, Isabelle Fajac, Tacjana Pressler, John Paul Clancy, Dorota Sands, Predrag Minic, Marco Cipolli, Ivanka Galeva, Amparo Solé, Alexandra L Quittner, Zhanna Jumadilova, Monika Ciesielska, Michael W Konstan, CLEAR-110 Study Group. J Cyst Fibros. 2021 Jun 15;S1569-1993(21)00164-8.doi: 10.1016/j.jcf.2021.05.013. Online ahead of print
Authors: Alienor Campredon, Enzo Battistella, Clémence Martin, Isabelle Durieu, Laurent Mely, Christophe Marguet, Chantal Belleguic, Marlène Murris-Espin, Raphaël Chiron, Annlyse Fanton, Stéphanie Bui, Martine Reynaud-Gaubert, Philippe Reix, Trieu-Nghi Hoang-Thi, Maria Vakalopoulou, Marie-Pierre Revel, Jennifer Da Silva, Pierre-Régis Burgel, Guillaume Chassagnon, French Cystic Fibrosis Reference Network Study Group. Eur Respir J. 2021 Nov 18;2101344.doi: 10.1183/13993003.01344-2021. Online ahead of print.
Authors: Agathe Lepissier, Charlotte Addy, Kate Hayes, Sabrina Noel, Stéphanie Bui, Pierre-Régis Burgel, Lieven Dupont, Olaf Eickmeier, Michael Fayon, Teresinha Leal, Carlos Lopes, Damian G Downey, Isabelle Sermet-Gaudelus. J Cyst Fibros. 2021 Nov 9;S1569-1993(21)02111-1. doi: 10.1016/j.jcf.2021.10.009. Online ahead of print.
Authors: Clémence Martin, Pierre-Régis Burgel. Eur Respir J. 2021 Oct 1;58(3):2101487.doi: 10.1183/13993003.01487-2021. Print 2021 Sep.
Authors: Jürg Barben, Carlo Castellani, Anne Munck, Jane C Davies, Karin M de Winter-de Groot, Silvia Gartner, Nataliya Kashirskaya, Barry Linnane, Sarah J Mayell, Susanna McColley, Chee Y Ooi, Marijke Proesmans, Clement L Ren, Danieli Salinas, Dorota Sands, Isabelle Sermet-Gaudelus, Olaf Sommerburg, Kevin W Southern, European CF Society Neonatal Screening Working Group (ECFS NSWG) J Cyst Fibros. 2021 Sep;20(5):810-819.doi: 10.1016/j.jcf.2020.11.006. Epub 2020 Nov 27.
Effects of a Partially Supervised Conditioning Program in Cystic Fibrosis: An International Multicenter Randomized Controlled Trial (ACTIVATE-CF)
Authors: Hebestreit H, Kriemler S, Schindler C, Stein L, Karila C, Urquhart DS, Orenstein DM, Lands LC, Schaeff J, Eber E, Radtke T, ACTIVATE-CF Study Working Group
Correction of CFTR function in intestinal organoids to guide treatment of cystic fibrosis.
Authors: Ramalho AS, Fürstová E, Vonk AM, Ferrante M, Verfaillie C, Dupont L, Boon M, Proesmans M, Beekman JM, Sarouk I, Vazquez Cordero C, Vermeulen F, De Boeck K; Belgian Organoid Project.
Lumacaftor-ivacaftor effects on cystic fibrosis-related liver involvement in adolescents with homozygous F508 del-CFTR.
Authors: Drummond D, Dana J, Berteloot L, Schneider-Futschik EK, Chedevergne F, Bailly-Botuha C, Nguyen-Khoa T, Cornet M, Le Bourgeois M, Debray D, Girard M, Sermet-Gaudelus I.Drummond D, et al.
Sweat Chloride Testing and Nasal Potential Difference (NPD) Are Primary Outcome Parameters in Treatment with Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators.
Authors: Sermet-Gaudelus I, Nguyen-Khoa T, Hatton A, Hayes K, Pranke I.Sermet-Gaudelus I, et al.
Antisense oligonucleotide-based drug development for Cystic Fibrosis patients carrying the 3849+10 kb C-to-T splicing mutation.
Authors: Oren YS, Irony-Tur Sinai M, Golec A, Barchad-Avitzur O, Mutyam V, Li Y, Hong J, Ozeri-Galai E, Hatton A, Leibson C, Carmel L, Reiter J, Sorscher EJ, Wilton SD, Kerem E, Rowe SM, Sermet-Gaudelus I, Kerem B.Oren YS, et al.
Airway Surface Liquid pH Regulation in Airway Epithelium Current Understandings and Gaps in Knowledge.
Authors: Zajac M, Dreano E, Edwards A, Planelles G, Sermet-Gaudelus I.Zajac M, et al.
Prior infection by seasonal coronaviruses, as assessed by serology, does not prevent SARS-CoV-2 infection and disease in children, France, April to June 2020.
Authors: Sermet-Gaudelus I, Temmam S, Huon C, Behillil S, Gajdos V, Bigot T, Lurier T, Chrétien D, Backovic M, Delaunay-Moisan A, Donati F, Albert M, Foucaud E, Mesplées B, Benoist G, Faye A, Duval-Arnould M, Cretolle C, Charbit M, Aubart M, Auriau J, Lorrot M, Kariyawasam D, Fertitta L, Orliaguet G, Pigneur B, Bader-Meunier B, Briand C, Enouf V, Toubiana J, Guilleminot T, van der Werf S, Leruez-Ville M, Eloit M.Sermet-Gaudelus I, et al.
New insights into structure and function of bis-phosphinic acid derivatives and implications for CFTR modulation.
Authors: Bitam S, Elbahnsi A, Creste G, Pranke I, Chevalier B, Berhal F, Hoffmann B, Servel N, Baatalah N, Tondelier D, Hatton A, Moquereau C, Faria Da Cunha M, Pastor A, Lepissier A, Hinzpeter A, Mornon JP, Prestat G, Edelman A, Callebaut I, Gravier-Pelletier C, Sermet-Gaudelus I.Bitam S, et al.
“Il faut continuer à poser des questions” patient reported outcome measures in cystic fibrosis: An anthropological perspective.
Authors: Coucke R, Chansard A, Bontemps V, Grenet D, Hubert D, Martin C, Lammertyn E, Bardin E, Bulteel V, Chedevergne F, Bourgeois ML, Burgel PR, Honore I, de Keyser H, Kirszenbaum M, de Carli P, Sermet-Gaudelus I, Hayes K; European Cystic Fibrosis Society-Clinical Trials Network Patient Advisory Group.Coucke R, et al.
Modulators of CFTR. Updates on clinical development and future directions.
Authors: Bardin E, Pastor A, Semeraro M, Golec A, Hayes K, Chevalier B, Berhal F, Prestat G, Hinzpeter A, Gravier-Pelletier C, Pranke I, Sermet-Gaudelus I.Bardin E, et al.
Pharmacokinetic and Pharmacodynamic Optimization of Antibiotic Therapy in Cystic Fibrosis Patients: Current Evidences, Gaps in Knowledge and Future Directions.
Authors: Magréault S, Roy C, Launay M, Sermet-Gaudelus I, Jullien V.Magréault S, et al.
Real-world assessment of LCI following lumacaftor-ivacaftor initiation in adolescents and adults with cystic fibrosis
Authors: Philippe Reix, Aurélie Tatopoulos, Iulia Ioan, Muriel Le Bourgeois, Stéphanie Bui, Marie Luce Choukroun, Katia Bessaci-Kabouya, Michele Gerardin, Plamen Bokov, Jennifer Da Silva, Jean-Louis Paillasseur, Pierre Regis Burgel, French Cystic Fibrosis Reference Network study group
2020
Update on Respiratory Fungal Infections in Cystic Fibrosis Lung Disease and after Lung Transplantation.
Authors: Renner S, Nachbaur E, Jaksch P, Dehlink E
Impact of COVID-19 on people with cystic fibrosis.
Authors: Carla Colombo, Pierre-Régis Burgel, Silvia Gartner, Silke van Koningsbruggen-Rietschel, Lutz Naehrlich, Isabelle Sermet-Gaudelus, Kevin W Southern.. DOI:10.1056/CAT.20.0080. Lancet Respir Med. 2020 May;8(5):e35-e36. doi: 10.1016/S2213-2600(20)30177-6. Epub 2020 Apr 15.
Use of a mobile application for self-management of pancreatic enzyme replacement therapy is associated with improved gastro-intestinal related quality of life in children with Cystic Fibrosis.
Authors: Boon M, Calvo-Lerma J, Claes I, Havermans T, Asseiceira I, Bulfamante A, Garriga M, Masip E, van Schijndel BAM, Fornes V, Barreto C, Colombo C, Crespo P, Vicente S, Janssens H, Hulst J, Witters P, Nobili R, Pereira L, Ruperto M, Van der Wiel E, Mainz JG, De Boeck K, Ribes-Koninckx C
Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID).
Authors: Barben J, Castellani C, Munck A, Davies JC, de Winter-de Groot KM, Gartner S, Kashirskaya N, Linnane B, Mayell SJ, McColley S, Ooi CY, Proesmans M, Ren CL, Salinas D, Sands D, Sermet-Gaudelus I, Sommerburg O, Southern KW; European CF Society Neonatal Screening Working Group (ECFS NSWG).
Urinary Exosomes of Patients with Cystic Fibrosis Unravel CFTR-Related Renal Disease.
Authors: Gauthier S, Pranke I, Jung V, Martignetti L, Stoven V, Nguyen-Khoa T, Semeraro M, Hinzpeter A, Edelman A, Guerrera IC, Sermet-Gaudelus I.
Reduced intestinal inflammation with Lumacaftor/Ivacaftor in adolescents with cystic fibrosis.
Authors: Tétard C, Mittaine M, Bui S, Beaufils F, Maumus P, Fayon M, Burgel P-R, Lamireau T, Delhaes L, Mas E, Enaud R
Clinical response to lumacaftor-ivacaftor in patients with cystic fibrosis according to baseline lung function.
Authors: Burgel P-R, Durieu I, Chiron R, Mely L, Prevotat A, Murris-Espin M, Porzio M, Abely M, Reix P, Marguet C, Macey J, Sermet-Gaudelus I, Corvol H, Bui S, Biouhee T, Hubert D, Munck A, Lemonnier L, Dehillotte C, Da Silva J, Paillasseur J-L, Martin C, French Cystic Fibrosis Reference Network study group
Protocol for Application, Standardization and Validation of the Forskolin-Induced Swelling Assay in Cystic Fibrosis Human Colon Organoids.
Authors: Vonk AM, van Mourik P, Ramalho AS, Silva IAL, Statia M, Kruisselbrink E, Suen SWF, Dekkers JF, Vleggaar FP, Houwen RHJ, Mullenders J, Boj SF, Vries R, Amaral MD, de Boeck K, van der Ent CK, Beekman JM.
Validation of Short- and Long-term Demographic Forecasts Using the Canadian Cystic Fibrosis Registry.
Authors: Martelli V, Sykes J, Burgel PR, Bellis G, Coriati A, Stanojevic S, Stephenson AL.
Getting neural about airway gland secretion
Authors: Amatngalim GD and Ribeiro CMP
R117H-CFTR Function and Response to VX-770 Correlate With mRNA and Protein Expression in Intestinal Organoids
Authors: Mourik PV, van Haaren P, Kruisselbrink E, Korkmaz C, Janssens HM, de Winter-de Groot KM, van der Ent CK, Hagemeijer MC, Beekman JM
Genetic Diagnosis in Practice: From Cystic Fibrosis to CFTR-related Disorders
Authors: Pagin A, Sermet-Gaudelus I, Burgel PR
2019
Cystic Fibrosis Diagnosis in Newborns, Children, and Adults.
Authors: Castellani C, Linnane B, Pranke I, Cresta F, Sermet-Gaudelus I, Peckham D.
Antimicrobial resistance in cystic fibrosis: A Delphi approach to defining best practices.
Authors: Zemanick E, Burgel PR, Taccetti G, Holmes A, Ratjen F, Byrnes CA, Waters VJ, Bell SC, VanDevanter DR, Stuart Elborn J, Flume PA; Antimicrobial Resistance International Working Group in Cystic Fibrosis
The future of cystic fibrosis care: a global perspective.
Authors: Bell SC, Mall MA, Gutierrez H, Macek M, Madge S, Davies JC, Burgel PR, Tullis E, Castaños C, Castellani C, Byrnes CA, Cathcart F, Chotirmall SH, Cosgriff R, Eichler I, Fajac I, Goss CH, Drevinek P, Farrell PM, Gravelle AM, Havermans T, Mayer-Hamblett N, Kashirskaya N, Kerem E, Mathew JL, McKone EF, Naehrlich L, Nasr SZ, Oates GR, O’Neill C, Pypops U, Raraigh KS, Rowe SM, Southern KW, Sivam S, Stephenson AL, Zampoli M, Ratjen F.
2025
Neutrophil-derived biomarkers in bronchiectasis: identifying a common therapeutic target.
Authors: Chalmers JD. Eur Respir J. 09/2025. doi: 10.1183/13993003.00081-2025.
Targeting neutrophil serine proteases in bronchiectasis.
Authors: Chalmers JD. Eur Respir J. 01/2025. doi: 10.1183/13993003.01050-2024.
Aspergillus Serologic Findings and Clinical Outcomes in Patients With Bronchiectasis: Data From the European Bronchiectasis Registry.
Authors: Pollock J. CHEST. 04/2025. doi: 10.1016/j.chest.2024.06.3843.
Efficacy of Serum Antiglycopeptidolipid Core IgA Antibody Test for Screening Nontuberculous Mycobacterial Pulmonary Disease in Bronchiectasis: A European Multicenter Cohort Study.
Authors: Choi H. CHEST. 05/2025. doi: 10.1016/j.chest.2024.10.029.
Use of inhaled corticosteroids in bronchiectasis: data from the European Bronchiectasis Registry (EMBARC).
Authors: Pollock J. Thorax. 05/2025. doi: 10.1136/thorax-2024-221825.
Sputum metagenomics in bronchiectasis reveals pan-European variation: an EMBARC-BRIDGE study.
Authors: Thng KX. Eur Respir J.. 08/2025. doi: 10.1183/13993003.00054-2025.
Symptoms, risk of future exacerbations, and response to long-term macrolide treatment in bronchiectasis: an observational study.
Authors: Sibila O. Lancet Respir Med. 10/2025. doi: 10.1016/S2213-2600(25)00160-2.
European Respiratory Society clinical practice guideline for the management of adult bronchiectasis.
Authors: Chalmers JD. Eur Respir J.. 12/2025. doi: 10.1183/13993003.01126-2025.
Molecular endotyping in people with bronchiectasis based on response to antibiotic treatment: iBEST study.
2024
EMBARCing on a new era for bronchiectasis: a review series for the Seventh World Bronchiectasis Conference.
Chalmers JD, Goeminne PC, Ringshausen FC.Eur Respir Rev. 2024 Jul 3;33(173):240124. doi: 10.1183/16000617.0124-2024. Print 2024 Jul.PMID: 38960616 Free PMC article.
Growing from common ground: nontuberculous mycobacteria and bronchiectasis.
Van Braeckel E, Bosteels C.Eur Respir Rev. 2024 Jul 3;33(173):240058. doi: 10.1183/16000617.0058-2024. Print 2024 Jul.PMID: 38960614 Free PMC article. Review.
Airway clearance management in people with bronchiectasis: data from the European Bronchiectasis Registry (EMBARC).
Spinou A, Hererro-Cortina B, Aliberti S, Goeminne PC, Polverino E, Dimakou K, Haworth CS, Loebinger MR, De Soyza A, Vendrell M, Burgel PR, McDonnell M, Sutharsan S, Škrgat S, Maiz-Carro L, Sibila O, Stolz D, Kauppi P, Bossios A, Hill AT, Clifton I, Crichton ML, Walker P, Menendez R, Borekci S, Obradovic D, Nowinski A, Amorim A, Torres A, Lorent N, Welte T, Blasi F, Jankovic Makek M, Shteinberg M, Boersma W, Elborn JS, Chalmers JD, Ringshausen FC; EMBARC Registry Collaborators.Eur Respir J. 2024 Jun 6;63(6):2301689. doi: 10.1183/13993003.01689-2023. Print 2024 Jun.PMID: 38609097 Free PMC article.
A systematic literature review of the clinical and socioeconomic burden of bronchiectasis.
Chalmers JD, Mall MA, McShane PJ, Nielsen KG, Shteinberg M, Sullivan SD, Chotirmall SH.Eur Respir Rev. 2024 Sep 4;33(173):240049. doi: 10.1183/16000617.0049-2024. Print 2024 Jul.PMID: 39231597 Free PMC article. Review.
Inhaled colistimethate sodium in patients with bronchiectasis and Pseudomonas aeruginosa infection: results of PROMIS-I and PROMIS-II, two randomised, double-blind, placebo-controlled phase 3 trials assessing safety and efficacy over 12 months.
Haworth CS, Shteinberg M, Winthrop K, Barker A, Blasi F, Dimakou K, Morgan LC, O’Donnell AE, Ringshausen FC, Sibila O, Thomson RM, Carroll KJ, Pontenani F, Castellani P, Chalmers JD; PROMIS trial investigators.Lancet Respir Med. 2024 Oct;12(10):787-798. doi: 10.1016/S2213-2600(24)00225-X. Epub 2024 Sep 10.PMID: 39270696 Free article. Clinical Trial.
Objective sputum colour assessment and clinical outcomes in bronchiectasis: data from the European Bronchiectasis Registry (EMBARC).
Aliberti S, Ringshausen FC, Dhar R, Haworth CS, Loebinger MR, Dimakou K, Crichton ML, De Soyza A, Vendrell M, Burgel PR, McDonnell M, Skrgat S, Maiz Carro L, de Roux A, Sibila O, Bossios A, van der Eerden M, Kauppi P, Wilson R, Milenkovic B, Menendez R, Murris M, Borekci S, Munteanu O, Obradovic D, Nowinski A, Amorim A, Torres A, Lorent N, Van Braeckel E, Altenburg J, Shoemark A, Shteinberg M, Boersma W, Goeminne PC, Elborn JS, Hill AT, Welte T, Blasi F, Polverino E, Chalmers JD; EMBARC Registry Investigators.Eur Respir J. 2024 Apr 18;63(4):2301554. doi: 10.1183/13993003.01554-2023. Print 2024 Apr.PMID: 38609095 Free PMC article.
Aspergillus Serologic Findings and Clinical Outcomes in Patients With Bronchiectasis: Data From the European Bronchiectasis Registry.
Pollock J, Goeminne PC, Aliberti S, Polverino E, Crichton ML, Ringshausen FC, Dhar R, Vendrell M, Burgel PR, Haworth CS, De Soyza A, De Gracia J, Bossios A, Rademacher J, Grünewaldt A, McDonnell M, Stolz D, Sibila O, van der Eerden M, Kauppi P, Hill AT, Wilson R, Amorim A, Munteanu O, Menendez R, Torres A, Welte T, Blasi F, Boersma W, Elborn JS, Shteinberg M, Dimakou K, Chalmers JD, Loebinger MR; EMBARC Registry Investigators.Chest. 2024 Oct 24:S0012-3692(24)05400-X. doi: 10.1016/j.chest.2024.06.3843. Online ahead of print.PMID: 39461553
Phenotypic and pathomechanistic overlap between tapasin and TAP deficiencies.
Elsayed A, von Hardenberg S, Atschekzei F, Graalmann T, Jänke C, Witte T, Ringshausen FC, Sogkas G.J Allergy Clin Immunol. 2024 Oct;154(4):1069-1075. doi: 10.1016/j.jaci.2024.06.003. Epub 2024 Jun 10.PMID: 38866210 Free article.
Evaluation of treatment response with serial CT in patients with non-tuberculous mycobacterial pulmonary disease.
Dettmer S, Heiß-Neumann M, Wege S, Maske H, Ringshausen FC, Joean O, Theissig N, Ewen R, Wacker F, Rademacher J.Eur Radiol. 2024 Aug 1. doi: 10.1007/s00330-024-10987-y. Online ahead of print.PMID: 39088042
[Management of adult bronchiectasis – Consensus-based Guidelines for the German Respiratory Society (DGP) e. V. (AWMF registration number 020-030)].
Ringshausen FC, Baumann I, de Roux A, Dettmer S, Diel R, Eichinger M, Ewig S, Flick H, Hanitsch L, Hillmann T, Koczulla R, Köhler M, Koitschev A, Kugler C, Nüßlein T, Ott SR, Pink I, Pletz M, Rohde G, Sedlacek L, Slevogt H, Sommerwerck U, Sutharsan S, von Weihe S, Welte T, Wilken M, Rademacher J, Mertsch P; Weitere beteiligte Wissenschaftliche Fachgesellschaften und Organisationen: Bundesverband der Pneumologen, Schlaf- und Beatmungsmediziner e. V. (BdP); Deutsche Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie e. V. (DGHNO-KHC); Deutsche Gesellschaft für Hygiene und Mikrobiologie e. V. (DGHM); Deutschen Gesellschaft für Immunologie e. V. (DGfI); Deutsche Gesellschaft für Infektiologie e. V. (DGI); Deutsche Gesellschaft für Rehabilitationswissenschaften e. V. (DGRW); Deutsche Gesellschaft für Thoraxchirurgie e. V. (DGT); Deutsche Deutsche Patientenliga Atemwegserkrankungen e. V. (DPLA); Deutsche Röntgen-Gesellschaft, Gesellschaft für Medizinische Radiologie e. V. (DRG); Deutscher Verband für Physiotherapie e. V. (ZVK); Gesellschaft für Pädiatrische Pneumologie e. V. (GPP); Deutsche Österreichische Gesellschaft für Pneumologie (ÖGP); Paul-Ehrlich Gesellschaft …
Clinical Efficacy of Serum Antiglycopeptidolipid Core IgA Antibody Test for Screening Nontuberculous Mycobacterial Pulmonary Disease in Bronchiectasis: A European Multicenter Cohort Study.
Choi H, Hughes C, Eke Z, Shuttleworth M, Shteinberg M, Polverino E, Goeminne PC, Welte T, Blasi F, Shoemark A, Long MB, Aliberti S, Haworth CS, Ringshausen FC, Loebinger MR, Lorent N, Chalmers JD.Chest. 2024 Oct 28:S0012-3692(24)05418-7. doi: 10.1016/j.chest.2024.10.029. Online ahead of print.PMID: 39490969 Free article.
Bronchiectasis and asthma: Data from the European Bronchiectasis Registry (EMBARC).
Authors: Polverino E, Dimakou K, Traversi L, Bossios A, Haworth CS, Loebinger MR, De Soyza A, Vendrell M, Burgel PR, Mertsch P, McDonnell M, Škrgat S, Maiz Carro L, Sibila O, van der Eerden M, Kauppi P, Hill AT, Wilson R, Milenkovic B, Menendez R, Murris M, Digalaki T, Crichton ML, Borecki S, Obradovic D, Nowinski A, Amorim A, Torres A, Lorent N, Welte T, Blasi F, Van Braeckel E, Altenburg J, Shoemark A, Shteinberg M, Boersma W, Elborn JS, Aliberti S, Ringshausen FC, Chalmers JD, Goeminne PC; EMBARC registry investigators. J Allergy Clin Immunol. 2024 Jun;153(6):1553-1562. doi: 10.1016/j.jaci.2024.01.027. Epub 2024 Feb 23. Erratum in: J Allergy Clin Immunol. 2024 Oct;154(4):1078. doi: 10.1016/j.jaci.2024.07.010. PMID: 38401857
2023
Bronchiectasis in Europe: data on disease characteristics from the European Bronchiectasis registry (EMBARC).
Authors: Chalmers JD, Polverino E, Crichton ML, Ringshausen FC, De Soyza A, Vendrell M, Burgel PR, Haworth CS, Loebinger MR, Dimakou K, Murris M, Wilson R, Hill AT, Menendez R, Torres A, Welte T, Blasi F, Altenburg J, Shteinberg M, Boersma W, Elborn JS, Goeminne PC, Aliberti S; EMBARC Registry Investigators. Lancet Respir Med. 2023;11(7):637-49. FI: 76,2.
The prevalence of bronchiectasis in patients with alpha-1 antitrypsin deficiency: initial report of EARCO.
Authors: Stockley RA, Pye A, De Soyza J, Turner AM, Miravitlles M; EARCO study investigators. Orphanet J Rare Dis. 2023;18(1):243. FI: 3,7. 1. Dhar R, Singh S, Talwar D, Murali Mohan BV, Tripathi SK, Swarnakar R, Trivedi S, Rajagopala S, D’Souza G, Padmanabhan A, Archana B, Mahesh PA, Ghewade B, Nair G, Jindal A, Jayadevappa GDH, Sawhney H, Sarmah KR, Saha K, Anantharaj S, Khanna A, Gami S, Shah A, Shah A, Dutt N, Garg H, Vyas S, Venugopal K, Prasad R, Aleemuddin NM, Karmakar S, Singh V, Jindal SK, Sharma S, Prajapat D, Chandrashekar S, Loebinger M, Mishra A, Blasi F, Ramanathan RP, Goeminne PC, Vasudev P, Shoemark A, Jayaraj BS, Kungwani R, Das A, Sawhney M, Polverino E, Welte T, Gulecha NS, Shteinberg M, Mangala A, Shah P, Chauhan NK, Jajodia N, Singhal A, Batra S, Hasan A, Aliberti S, Crichton ML, Limaye S, Salvi S, Chalmers JD; EMBARC-India study group. Clinical outcomes of bronchiectasis in India: data from the EMBARC/Respiratory Research Network of India registry. Eur Respir J. 2023;61(1):2200611. FI: 24,9.
Transforming clinical research and science in bronchiectasis: EMBARC3, a European Respiratory Society Clinical Research Collaboration.
Authors: Chalmers JD, Aliberti S, Altenburg J, Blasi F, Clarke C, Chotirmall SH, Crichton ML, Dhar R, Goeminne P, Haworth C, Loebinger MR, Lorent N, Polverino E, Ringshausen FC, Shoemark A, Shteinberg M, Sibila O, Spinou A, Welte T. Eur Respir J. 2023;61(6):2300769. FI: 24,9.
2019
Recommendations for travelling with bronchiectasis: a joint ELF/EMBARC/ERN-Lung collaboration.
Authors: Shteinberg M, Crossley B, Lavie T, Nadler S, Boyd J, Ringshausen FC, Aksamit T, Chalmers JD, Goeminne P
2018
The European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) ERS Clinical Research Collaboration.
Authors: Aliberti S, Polverino E, Chalmers JD, Altenburg J, Shteinberg M, Goeminne PC, Welte T, Shoemark A, Almagro M, Blasi F; EMBARC Clinical Research Collaboration.Cross-infection risk in patients with bronchiectasis: a position statement from the European Bronchiectasis Network (EMBARC), EMBARC/ELF patient advisory group and European Reference Network (ERN-Lung) Bronchiectasis Network.
Authors: Chalmers JD, Ringshausen FC, Harris B, Elborn JS, Posthumus A, Haworth CS, Pilkington N, Polverino E, Ruddy T, Aliberti S, Goeminne PC, Winstanley C, De Soyza A.2025
Co-targeting of VEGFR2 and PD-L1 promotes survival and vasculature normalization in pleural mesothelioma.
Authors: Rovers S. Oncoimmunology. 12/2025. doi: 10.1080/2162402X.2025.2512104.
2024
A randomised phase II study of extended pleurectomy/decortication preceded or followed by chemotherapy in patients with early-stage pleural mesothelioma: EORTC 1205.
Raskin J, Surmont V, Maat APWM, Yahia M, Burgers SA, Pretzenbacher Y, De Ryck F, Cornelissen R, Klomp HM, Oliveira A, Aerts J, Baas P, Marreaud S, Van Meerbeeck JP, Van Schil PEY.Eur Respir J. 2024 Jun 28;63(6):2302114. doi: 10.1183/13993003.02114-2023. Print 2024 Jun.PMID: 38843916 Free PMC article. Clinical Trial.
2022
Are anaplastic lymphoma kinase (ALK) and O6-methylguanine-DNA methyltransferase (MGMT) promoter methylation driver biomarkers of pulmonary neuroendocrine tumors (NETs) and carcinomas (NECs)?
Authors: Hiddinga B, Zwaenepoel K, Janssens A, Van Meerbeeck J, Pauwels P.Oncotarget. 2022 Jun 1;13:800-809. doi: 10.18632/oncotarget.28240. eCollection 2022.PMID: 35677534 Free PMC article.
Anetumab ravtansine versus vinorelbine in patients with relapsed, mesothelin-positive malignant pleural mesothelioma (ARCS-M): a randomised, open-label phase 2 trial.
Authors: Kindler HL, Novello S, Bearz A, Ceresoli GL, Aerts JGJV, Spicer J, Taylor P, Nackaerts K, Greystoke A, Jennens R, Calabrò L, Burgers JA, Santoro A, Cedrés S, Serwatowski P, Ponce S, Van Meerbeeck JP, Nowak AK, Blumenschein G Jr, Siegel JM, Kasten L, Köchert K, Walter AO, Childs BH, Elbi C, Hassan R, Fennell DA.Lancet Oncol. 2022 Apr;23(4):540-552. doi: 10.1016/S1470-2045(22)00061-4.PMID: 35358455
2021
Clinical utility of diagnostic biomarkers in malignant pleural mesothelioma: a systematic review and meta-analysis.
Authors: Eline Schillebeeckx, Jan P van Meerbeeck, Kevin Lamote Eur Respir Rev. 2021 Nov 17;30(162):210057. doi: 10.1183/16000617.0057-2021. Print 2021 Dec 31.
Recent developments in the treatment of small cell lung cancer.
Authors: Birgitta I Hiddinga, Jo Raskin, Annelies Janssens, Patrick Pauwels, Jan P Van Meerbeeck. Eur Respir Rev. 2021 Jul 13;30(161):210079.doi: 10.1183/16000617.0079-2021. Print 2021 Sep 30.
Safety of G-CSF with concurrent chemo-radiotherapy in limited-stage small cell lung cancer – Secondary analysis of the randomised phase 3 CONVERT trial.
Authors: Gomes F, Faivre-Finn C, Mistry H, Bezjak A, Pourel N, Fournel P, Van Meerbeeck J, Blackhall F
2020
Volatile organic compounds in human matrices as lung cancer biomarkers: A systematic review.
Authors: Janssens E, van Meerbeeck JP, Lamote K
CheckMate 171: A phase 2 trial of nivolumab in patients with previously treated advanced squamous non-small cell lung cancer, including ECOG PS 2 and elderly populations
Authors: Felip E, Ardizzoni A, Ciuleanu T, Cobo M, Laktionov K, Szilasi M, Califano R, Carcereny E, Griffiths R, Paz-Ares L, Duchnowska R, Garcia MA, Isla D, Jassem J, Appel W, Milanowski J, Van Meerbeeck JP, Wolf J, Li A, Acevedo A, Popat S
2019
Tumour Treating Fields in combination with pemetrexed and cisplatin or carboplatin as first-line treatment for unresectable malignant pleural mesothelioma (STELLAR): a multicentre, single-arm phase 2 trial.
Authors: Ceresoli GL, Aerts JG, Dziadziuszko R, Ramlau R, Cedres S, van Meerbeeck JP, Mencoboni M, Planchard D, Chella A, Crinò L, Krzakowski M, Rüssel J, Maconi A, Gianoncelli L, Grosso F.
Association between surgical volume and post-operative mortality and survival after surgical resection in lung cancer in Belgium: A population-based study.
Authors: Schillemans V, Vrijens F, De Gendt C, Robays J, Silversmit G, Verleye L, Camberlin C, Dubois C, Stordeur S, Wauters I, Van Meerbeeck JP, Van Eycken E, De Leyn P.2025
Buddy, bystander or betrayer: B cells in lymphoid aggregates in AATD emphysema.
Authors: Khedoe, van ‘t Wout. Eur Respir J. 01/2025. doi: 10.1183/13993003.02163-2024. PMID: 39746767 NO
Clinical features in patients with severe Alpha-1 antitrypsin deficiency due to rare genotypes.
Authors: Ferrarotti I. Pulmonology. 12/2025. doi: 10.1080/25310429.2024.2429911.
Longitudinal Evaluation of Individuals With Severe Alpha-1 Antitrypsin Deficiency (Pi∗ZZ Genotype).
Authors: Fromme M, Payancé A, Mandorfer M, Thorhauge KH, Pons M, Miravitlles M, Stolk J, van Hoek B, Stirnimann G, Frankova S, Sperl J, Kremer AE, Burbaum B, Schrader C, Kadioglu A, Walkenhaus M, Schneider CV, Klebingat F, Balcar L, Kappe NN, … Strnad P. Gastroenterology. 168/2025. doi: 10.1053/j.gastro.2024.10.010.
Pathologists’ experience and routine practice in high-volume lung transplant centers: An international survey.
Authors: Calabrese F, Hwang DM, Lunardi F, Iannaco R, Adam J, Akyürek LM, Adam B, Jan B, Berry G, D’Sa L, Fabre A, Fattori A, Fishbein GA, Fredenburg KM, Romero TH, Husain AN, Jones K, Kern I, Lin CY, Louis R, … Levine DJ. JHLT Open. 11/2025. doi: 10.1016/j.jhlto.2025.100415.
Seasonal Changes in Circulating Alpha-1 Antitrypsin Are Altered by Sex and Genetic Deficiency.
Authors: Leacy EJ, Fraughen DD, Heeney RC, Casey M, Sherlock M, O’Reilly MW, Murphy MP, Curtis AM, Carroll TP, McElvaney NG. Am J Respir Crit Care Med. 211/2025. doi: 10.1164/rccm.202506-1341RL.
Lung disease in never-smokers with severe α1-antitrypsin deficiency: the EARCO Registry.
Authors: Premuda C. ERJ Open Res. 11/2025. doi: 10.1183/23120541.01279-2024.
2024
Assessment and monitoring of lung disease in patients with severe alpha 1 antitrypsin deficiency: a european delphi consensus of the EARCO group.
Miravitlles M, Turner AM, Sucena M, Mornex JF, Greulich T, Wencker M, McElvaney NG.Respir Res. 2024 Aug 19;25(1):318. doi: 10.1186/s12931-024-02929-5.PMID: 39160517 Free PMC article.
Alcohol consumption and liver phenotype of individuals with alpha-1 antitrypsin deficiency.
Fromme M, Schneider CV, Guldiken N, Amzou S, Luo Y, Pons M, Genesca J, Miravitlles M, Thorhauge KH, Mandorfer M, Waern J, Schneider KM, Sperl J, Frankova S, Bartel M, Zimmer H, Zorn M, Krag A, Turner A, Trautwein C, Strnad P.Liver Int. 2024 Oct;44(10):2660-2671. doi: 10.1111/liv.16044. Epub 2024 Jul 19.PMID: 39031304
Longitudinal Evaluation of Individuals With Severe Alpha-1 Antitrypsin Deficiency (Pi∗ZZ Genotype).
Fromme M, Payancé A, Mandorfer M, Thorhauge KH, Pons M, Miravitlles M, Stolk J, van Hoek B, Stirnimann G, Frankova S, Sperl J, Kremer AE, Burbaum B, Schrader C, Kadioglu A, Walkenhaus M, Schneider CV, Klebingat F, Balcar L, Kappe NN, Schaefer B, Chorostowska-Wynimko J, Aigner E, Gensluckner S, Striedl P, Roger P, Ryan J, Roche S, Vögelin M, Ala A, Bantel H, Verbeek J, Mariño Z, Praktiknjo M, Gevers TJG, Reuken PA, Berg T, George J, Demir M, Bruns T, Trautwein C, Zoller H, Trauner M, Genesca J, Griffiths WJ, Clark V, Krag A, Turner AM, McElvaney NG, Strnad P.Gastroenterology. 2025 Feb;168(2):367-381. doi: 10.1053/j.gastro.2024.10.010. Epub 2024 Oct 15.PMID: 39414159 Free article
Sex-Differences in Alpha-1 Antitrypsin Deficiency: Data From the EARCO Registry.
Ersöz H, Torres-Durán M, Turner AM, Tanash H, Rodríguez García C, Corsico AG, López-Campos JL, Miravitlles M, Clarenbach CF, Chapman KR, Hernández Pérez JM, Guimarães C, Bartošovská E, Greulich T, Barrecheguren M, Koczulla AR, Höger P, Olivares Rivera A, Herth F, Trudzinski FC; EARCO study investigators.Arch Bronconeumol. 2025 Jan;61(1):22-30. doi: 10.1016/j.arbres.2024.06.019. Epub 2024 Jul 9.PMID: 39068055 Free article. English, Spanish.
Clinical features in patients with severe Alpha-1 antitrypsin deficiency due to rare genotypes.
Ferrarotti I, Piloni D, Filosa A, Ottaviani S, Barzon V, Balderacchi AM, Corda L, Seebacher C, Magni S, Mariani F, Baderna P, Confalonieri P, Iannacci L, Mancinelli S, Putignano P, Albera C, Stella GM, Monti MC, Corsico AG.Pulmonology. 2025 Dec 31;31(1):2429911. doi: 10.1080/25310429.2024.2429911. Epub 2024 Dec 3.PMID: 39624947 Free article.
Alpha-1 Antitrypsin Augmentation and the Liver Phenotype of Adults With Alpha-1 Antitrypsin Deficiency (Genotype Pi∗ZZ).
Authors: Fromme M, Hamesch K, Schneider CV, Mandorfer M, Pons M, Thorhauge KH, Pereira V, Sperl J, Frankova S, Reichert MC, Benini F, Burbaum B, Kleinjans M, Amzou S, Rademacher L, Bewersdorf L, Verbeek J, Nevens F, Genesca J, Miravitlles M, Nuñez A, Schaefer B, Zoller H, Janciauskiene S, Waern J, Oliveira A, Maia L, Simões C, Mahadeva R, Fraughen DD, Trauner M, Krag A, Lammert F, Bals R, Gaisa NT, Aigner E, Griffiths WJ, Denk H, Teumer A, McElvaney NG, Turner AM, Trautwein C, Strnad P. Clin Gastroenterol Hepatol. 2024 Feb;22(2):283-294.e5. doi: 10.1016/j.cgh.2023.08.038. Epub 2023 Sep 15.
PMID: 37716616
2023
Alpha-1 antitrypsin (AAT) augmentation and the liver phenotype of adults with AAT deficiency (genotype Pi*ZZ)
Authors: Federica Benini.
Clin Gastroenterol Hepatol. 2023:S1542-3565(23)00708-5. doi: 10.1016/j.cgh.2023.08.038
Comparison among populations with severe and intermediate alpha1-antitrypsin deficiency and chronic obstructive pulmonary disease.
Authors: Luciano Corda Minerva Med. 2023 Apr 6. doi: 10.23736/S0026-4806.22.08266-0. PMID: 37021471
Distribution of alpha1 antitrypsin rare alleles in six countries: Results from the Progenika diagnostic network.
Authors: Lopez-Campos JL, Rapun N, Czischke K, Jardim JR, Acquier MF, Munive AA, Günen H, Drobnic E, Miravitlles M, Osaba. Hum Genomics. 2023;17(1):48. FI: 4,5.
Characteristics of individuals with alpha-1 antitrypsin deficiency from Northern and Southern European countries: EARCO international registry.
Authors: Miravitlles M, Turner AM, Torres-Duran M, Tanash H, Rodríguez-García C, López-Campos JL, Chlumsky J, Guimaraes C, Rodríguez-Hermosa JL, Corsico A, Martinez-González C, Hernández-Pérez JM, Bustamante A, Parr DG, Casas-Maldonado F, Hecimovic A, Janssens W, Lara B, Barrecheguren M, González C, Stolk J, Esquinas C, Clarenbach CF; EARCO study investigators. Eur Respir J. 2023;61(3):2201949. FI: 24,9.
2021
Long-term effect of α1-antitrypsin augmentation therapy on the decline of FEV1 in deficient patients: an analysis of the AIR database
Authors: Iris G.M. Schouten, Marise J. Kasteleyn, Roula Tsonaka, Robert Bals, Alice C. Turner, Ilaria Ferrarotti, Angelo G. Corsico, Beatriz Lara, Marc Miravitlles, Robert A. Stockley, Jan Stolk
Polymerization of misfolded Z alpha-1 antitrypsin protein lowers CX3CR1 expression in human PBMCs
Authors: Srinu Tumpara, Matthias Ballmaier, Sabine Wrenger, Mandy König, Matthias Lehmann, Ralf Lichtinghagen, Beatriz Martinez-Delgado, Elena Korenbaum, David DeLuca, Nils Jedicke, Tobias Welte, Malin Fromme, Pavel Strnad, Jan Stolk, Sabina Janciauskiene
The Course of AαVal541 as a Proteinase 3 Specific Neo-Epitope after Alpha-1-Antitrypsin Augmentation in Severe Deficient Patients
Authors: Iris G. M. Schouten, Richard A. Mumford, Dirk Jan A. R. Moes, Pieter S. Hiemstra, and Jan Stolk
2020
Research priorities in alpha-1-antitrypsin deficiency: results of a patients’ and healthcare providers’ international survey from the EARCO Clinical Research Collaboration
Authors: Barrecheguren M, O’Hara K, Wilkens M, Boyd J, Kolda E, Lara B, Chorostowska-Wynimko J, Ferrarotti I, Chlumský J, Clarenbach C, Greulich T, Miravitlles M and Sucena M, on behalf of EARCO
New Patient-Centric Approaches to the Management of Alpha-1 Antitrypsin Deficiency
Authors: Chorostowska-Wynimko J, Barrecheguren M, Ferrarotti I, Greulich T, Sandhaus RA, and Campos M
2019
Efficacy and safety of inhaled α1-antitrypsin in patients with severe α1-antitrypsin deficiency and frequent exacerbations of COPD.
Authors: Stolk J, Tov N, Chapman KR, Fernandez P, MacNee W, Hopkinson NS, Piitulainen E, Seersholm N, Vogelmeier CF, Bals R, McElvaney G, Stockley RA.
The European Alpha-1 Research Collaboration (EARCO): a new ERS Clinical Research Collaboration to promote research in alpha-1 antitrypsin deficiency.
Authors: Miravitlles M, Chorostowska-Wynimko J, Ferrarotti I, McElvaney NG, O’Hara K, Stolk J, Stockley RA, Turner A, Wilkens M, Greulich T; EARCO Clinical Research Collaboration; Members of the EARCO Clinical Research Collaboration.
Liver Fibrosis and Metabolic Alterations in Adults With alpha-1-antitrypsin Deficiency Caused by the Pi*ZZ Mutation.
Authors: Hamesch K, Mandorfer M, Pereira VM, Moeller LS, Pons M, Dolman GE, Reichert MC, Schneider CV, Woditsch V, Voss J, Lindhauer C, Fromme M, Spivak I, Guldiken N, Zhou B, Arslanow A, Schaefer B, Zoller H, Aigner E, Reiberger T, Wetzel M, Siegmund B, Simões C, Gaspar R, Maia L, Costa D, Bento-Miranda M, van Helden J, Yagmur E, Bzdok D, Stolk J, Gleiber W, Knipel V, Windisch W, Mahadeva R, Bals R, Koczulla R, Barrecheguren M, Miravitlles M, Janciauskiene S, Stickel F, Lammert F, Liberal R, Genesca J, Griffiths WJ, Trauner M, Krag A, Trautwein C, Strnad P; European Alpha1-Liver Study Group.
2025
Multiple breath washout in primary ciliary dyskinesia: a systematic review of the literature.
Ματτηαιοθ ΑΜ, ετ αλ. European Respiratory Reviews. 08/2025. doi: 10.1183/16000617.0002-2025.
The CARMUCI Study Design: A Double-Blind, Cross-Over Sham-Controlled Trial of Indoor Air Purification in People With Cystic Fibrosis and Primary Ciliary Dyskinesia.
Kakkoura M, et al. Pediatric Pulmonology. 02/2026. doi: doi: 10.1002/ppul.71514.
Natural variability of lung function in primary ciliary dyskinesia: longitudinal analysis from the PROVALF-PCD cohort.
Kewei Zhang et al. ERJ Open Res .. 06/2025. doi: 10.1183/23120541.01115-2024.
International consensus statement on routine blood testing in primary ciliary dyskinesia.
Authors: Altaraih S. ERJ Open Res. 01/2025. doi: 01071-2024.
National survey on pediatric respiratory physiotherapy units: primary ciliary dyskinesia and non-CF bronchiectasis.
Authors: Tani B. Ital J Pediatr. 01/2025. doi: 6;51(1):67.
Physiotherapy in Primary Ciliary Dyskinesia.
Authors: Krämer C. Pneumologie. 08/2025. doi: 10.1055/a-2502-8151
European Respiratory Society and American Thoracic Society guidelines for the diagnosis of primary ciliary dyskinesia.
Authors: Shoemark A et al. The European Respiratory Journal. 12/2025. doi: 10.1183/13993003.00745-2025.
Editorial: Distinct phenotype but same genotype: hints for the diversity of phenotypes in ciliopathies.
Authors: Sayer J, Omran H, Zietkiewicz E. 01/2025. doi: doi: 10.3389/fmolb.2025.1753098.
Limitations of PICADAR as a diagnostic predictive tool for primary ciliary dyskinesia.
Authors: Schramm A, Raidt J, Riepenhausen S, Nygaard CMT, Tenardi-Wenge R, Qvist T, Witt P, Storck M, Olbrich H, Nielsen KG, Omran H.01/2025. doi: doi: 10.3389/fmolb.2025.1691758.
Clinical, phenotype and genotype correlations in primary ciliary dyskinesia suspected children in Egypt.
Authors: Hoda Rizk, Rim Hjeij, Mohammad Al-Haggar, Bernd Dworniczak, Dominik Otto Heike Olbrich, Engy Osman, Heymut Omran, and Tarek Eldesoky
02/2025. doi: doi: 10.3389/fmolb.2025.1641739.
Primary ciliary dyskinesia phenotypes and correlation with genotype.
Authors: Horani A, Wee W, Omran H, Ferkol T. 01/2025. doi: doi: 10.1097/MCP.0000000000001212.
Natural variability of lung function in primary ciliary dyskinesia: longitudinal analysis from the PROVALF-PCD cohort.
Authors: Zhang et al. Front. Pharmacol. 01/2025. doi: doi: 10.1183/23120541.01115-2024.
International consensus statement on routine blood testing in primary ciliary dyskinesia.
Authors: Altaraihi et al. ERJ Open Res. 01/2025. doi: doi: 10.1183/23120541.01071-2024.
Empowering limited-resource countries: collaborating with expert centers for diagnosis of primary ciliary dyskinesia.
Authors: Kalyoncu et al. 01/2025. doi: doi: 10.3389/fmolb.2025.1547152.
Pathogenic KIAA0586/TALPID3 variants are associated with defects in primary and motile cilia.
Authors: Taudien JE, Bracht D, Olbrich H, Swirski S, D’Abrusco F, Van der Zwaag B, Möller M, Lücke T, Teig N, Lindberg U, Wohlgemuth K, Wallmeier J, Blanque A, Gatsogiannis C, George S, Jüschke C, Owczarek-Lipska M, Veer D, Kroes HY, Valente EM, Korenke GC, Omran H, Neidhardt J. doi:10.1016/j.isci.2024.111670.
Cyclin O controls entry into the cell-cycle variant required for multiciliated cell differentiation.
Authors: Damaa et al. 01/2025. doi: doi: 10.1016/j.celrep.2024.115117.
Long-Term Lung Function and Pseudomonas aeruginosa Infection in Genotyped Primary Ciliary Dyskinesia.
Authors: Mathias G. Holgersen , June K. Marthin , Johanna Raidt , Tavs Qvist , Helle K. Johansen , Heymut Omran , Kim G. Nielsen. American Thoracic Society. 01/2025. doi: doi: 10.1513/AnnalsATS.202404-340OC.
Components of the Metabolic Syndrome Are Associated With Decline in FEV₁ and With All-Cause Mortality in COPD.
Authors: Wijnant SRA. Respir Med. 05/2025. doi: 10.1016/j.rmed.2025.108329.
2024
Associations between respiratory pathogens and lung function in primary ciliary dyskinesia: cross-sectional analysis from the PROVALF-PCD cohort
Authors: Rubbo B, Kant A, Zhang K, Allegorico A, Basilicata S, Boon M, Borrelli M, Calogero C, Carr SB, Carroll M, Constant C, Castillo Corullón S, Corvol H, Cutrera R, Dillenhöfer S, Emiralioglu N, Eralp E, Eryilmaz Polat S, Gardner L, Gokdemir Y, Harris A, Hogg C, Karadag B, Kobbernagel H, Koerner-Rettberg C, Kouis P, Lorent N, Marcou M, Mathin JK, Martinu V, Moreno-Galdó A, Morgan L, Nielsen KG, Omran H, Ozcelik U, Pohunek P, Raidt J, Robinson P, Rovira-Amigo S, Santamaria F, Schlegtendal A, Tamalet A, Thouvenin G, Ullmann N, Walker W, Yiallouros P, Kuehni CE, Latzin P, Beydon N, Lucas JS. ERJ Open Res. 2024 Sep 30;10(5):00253-2024. doi: 10.1183/23120541.00253-2024. PMID: 39351386; PMCID: PMC11440376.
[Primary ciliary dyskinesia].
Authors: Raidt J, Staar BO, Omran H, Ringshausen FC.Inn Med (Heidelb). 2024 Jun;65(6):545-559. doi: 10.1007/s00108-024-01726-y. Epub 2024 May 27.PMID: 38801438 Review. German.
Analyses of 1236 genotyped primary ciliary dyskinesia individuals identify regional clusters of distinct DNA variants and significant genotype-phenotype correlations.
Raidt J, Riepenhausen S, Pennekamp P, Olbrich H, Amirav I, Athanazio RA, Aviram M, Balinotti JE, Bar-On O, Bode SFN, Boon M, Borrelli M, Carr SB, Crowley S, Dehlink E, Diepenhorst S, Durdik P, Dworniczak B, Emiralioğlu N, Erdem E, Fonnesu R, Gracci S, Große-Onnebrink J, Gwozdziewicz K, Haarman EG, Hansen CR, Hogg C, Holgersen MG, Kerem E, Körner RW, Kötz K, Kouis P, Loebinger MR, Lorent N, Lucas JS, Maj D, Mall MA, Marthin JK, Martinu V, Mazurek H, Mitchison HM, Nöthe-Menchen T, Özçelik U, Pifferi M, Pogorzelski A, Ringshausen FC, Roehmel JF, Rovira-Amigo S, Rumman N, Schlegtendal A, Shoemark A, Sperstad Kennelly S, Staar BO, Sutharsan S, Thomas S, Ullmann N, Varghese J, von Hardenberg S, Walker WT, Wetzke M, Witt M, Yiallouros P, Zschocke A, Ziętkiewicz E, Nielsen KG, Omran H.Eur Respir J. 2024 Aug 8;64(2):2301769. doi: 10.1183/13993003.01769-2023. Print 2024 Aug.PMID: 38871375 Free PMC article.
A BEAT-PCD consensus statement: a core outcome set for pulmonary disease interventions in primary ciliary dyskinesia.
Kos R, Goutaki M, Kobbernagel HE, Rubbo B, Shoemark A, Aliberti S, Altenburg J, Anagnostopoulou P, Athanazio RA, Beydon N, Dell SD, Emiralioglu N, Ferkol TW, Loebinger MR, Lorent N, Maître B, Marthin J, Morgan LC, Nielsen KG, Ringshausen FC, Shteinberg M, Tiddens HAWM, Maitland-Van der Zee AH, Chalmers JD, Lucas JSA, Haarman EG.ERJ Open Res. 2024 Jan 8;10(1):00115-2023. doi: 10.1183/23120541.00115-2023. eCollection 2024 Jan.PMID: 38196895 Free PMC article.
Long-Term Lung Function and Pseudomonas aeruginosa Infection in Genotyped Primary Ciliary Dyskinesia.
Holgersen MG, Marthin JK, Raidt J, Qvist T, Johansen HK, Omran H, Nielsen KG.Ann Am Thorac Soc. 2024 Oct 24. doi: 10.1513/AnnalsATS.202404-340OC. Online ahead of print.PMID: 39447114
Association between upper and lower respiratory disease among patients with primary ciliary dyskinesia: an international study.
Authors: Lam, Y. T., Papon, J. F., Alexandru, M., Anagiotos, A., Armengot, M., Boon, M., Burgess, A., Calmes, D., Crowley, S., Dheyauldeen, S. A. D., Emiralioglu, N., Erdem Eralp, E., van Gogh, C., Gokdemir, Y., Haarman, E. G., Harris, A., Hayn, I., Ismail-Koch, H., Karadag, B., Kempeneers, C., Goutaki, M. (2024). ERJ open research, 10(2), 00932-2023. https://doi.org/10.1183/23120541.00932-2023
A novel mutation in GAS8 gene associated with chronic rhinosinusitis with nasal polyposis in a case of primary ciliary dyskinesia: a case report
Authors: Artesani MC, Santarsiero S, Sitzia E, Lepri FR, Magliozzi M, Majo F, Ullmann N, Stracuzzi A, Novelli A, Cristalli G, Fiocchi A.. Front Pediatr. 2024 May 30;12:1345265.
Cyclin O controls entry into the cell-cycle variant required for multiciliated cell differentiation.
Authors: Faucourt M, Delgehyr N, Goh KJ, Lu H, Tan EK, James CT, Faucon C, Mitri R, Bracht DC, Bingle CD, Dunn NR, Arnold SJ, Zaragosi LE, Barbry P, Koszul R, Omran H, Gil-Gómez G, Escudier E, Legendre M, Roy S, Spassky N, Meunier A. Cell Rep. 2025 Jan 28;44(1):115117. doi: 10.1016/j.celrep.2024.115117. Epub 2024 Dec 30. PMID: 39740663
Pathogenic variants in <i>CFAP46</i>, <i>CFAP54</i>, <i>CFAP74</i> and <i>CFAP221</i> cause primary ciliary dyskinesia with a defective C1d projection of the central apparatus.
Authors: Wohlgemuth K, Hoersting N, Koenig J, Loges NT, Raidt J, George S, Cindrić S, Schramm A, Biebach L, Lay S, Dougherty GW, Olbrich H, Pennekamp P, Dworniczak B, Omran H. Eur Respir J. 2024 Dec 12;64(6):2400790. doi: 10.1183/13993003.00790-2024. PMID: 39362668; PMCID: PMC11651342.
Primary Ciliary Dyskinesia Associated Disease-Causing Variants in <i>CCDC39</i> and <i>CCDC40</i> Cause Axonemal Absence of Inner Dynein Arm Heavy Chains DNAH1, DNAH6, and DNAH7.
Authors: Wilken A, Höben IM, Wolter A, Loges NT, Olbrich H, Aprea I, Dworniczak B, Raidt J, Omran H. Cells. 2024 Jul 15;13(14):1200. doi: 10.3390/cells13141200. PMID: 39056782; PMCID: PMC11274998.
Novel pathogenic variants of <i>DNAH5</i> associated with clinical and genetic spectra of primary ciliary dyskinesia in an Arab population.
Authors: Al-Mutairi DA, Alsabah BH, Pennekamp P, Omran H. Front Genet. 2024 Jul 10;15:1396797. doi:10.3389/fgene.2024.1396797. PMID: 39045318; PMCID: PMC11264286.
Recessively Inherited Deficiency of Secreted WFDC2 (HE4) Causes Nasal Polyposis and Bronchiectasis.
Authors: Dougherty GW, Ostrowski LE, Nöthe-Menchen T, Raidt J, Schramm A, Olbrich H, Yin W, Sears PR, Dang H, Smith AJ, Beule AG, Hjeij R, Rutjes N, Haarman EG, Maas SM, Ferkol TW, Noone PG, Olivier KN, Bracht DC, Barbry P, Zaragosi LE, Fierville M, Kliesch S, Wohlgemuth K, König J, George S, Loges NT, Ceppe A, Markovetz MR, Luo H, Guo T, Rizk H, Eldesoky T, Dahlke K, Boldt K, Ueffing M, Hill DB, Pang YP, Knowles MR, Zariwala MA, Omran H.. Am J Respir Crit Care Med. 2024 Jul 1;210(1):63-76. doi: 10.1164/rccm.202308-1370OC. PMID:38626355; PMCID: PMC11197063.
Biallelic Variants in <i>MNS1</i> Are Associated with Laterality Defects and Respiratory Involvement. Authors: Hjeij R, Leslie J, Rizk H, Dworniczak B, Olbrich H, Raidt J, Bode SFN, Gardham A, Stals K, Al-Haggar M, Osman E, Crosby A, Eldesoky T, Baple E, Omran H. Cells. 2024 Jun 11;13(12):1017. doi: 10.3390/cells13121017. PMID: 38920647; PMCID: PMC11202006.
Ciliopathy patient variants reveal organelle-specific functions for TUBB4B in axonemal microtubules.
Authors: Dodd DO, Mechaussier S, Yeyati PL, McPhie F, Anderson JR, Khoo CJ, Shoemark A, Gupta DK, Attard T, Zariwala MA, Legendre M, Bracht D, Wallmeier J, Gui M, Fassad MR, Parry DA, Tennant PA, Meynert A, Wheway G, Fares-Taie L, Black HA, Mitri-Frangieh R, Faucon C, Kaplan J, Patel M, McKie L, Megaw R, Gatsogiannis C, Mohamed MA, Aitken S, Gautier P, Reinholt FR, Hirst RA, O’Callaghan C, Heimdal K, Bottier M, Escudier E, Crowley S, Descartes M, Jabs EW, Kenia P, Amiel J, Bacci GM, Calogero C, Palazzo V, Tiberi L, Blümlein U, Rogers A, Wambach JA, Wegner DJ, Fulton AB, Kenna M, Rosenfeld M, Holm IA, Quigley A, Hall EA, Murphy LC, Cassidy DM, von Kriegsheim A; Scottish Genomes Partnership16; Genomics England Research Consortium45; Undiagnosed Diseases Network46; Papon JF, Pasquier L, Murris MS, Chalmers JD, Hogg C, Macleod KA, Urquhart DS, Unger S, Aitman TJ, Amselem S, Leigh MW, Knowles MR, Omran H, Mitchison HM, Brown A, Marsh JA, Welburn JPI, Ti SC, Horani A, Rozet JM, Perrault I, Mill P. Science. 2024 Apr 26;384(6694):eadf5489. doi:10.1126/science.adf5489. Epub 2024 Apr 26. Erratum in: Science. 2024 May 10;384(6696):eadq2178. doi: 10.1126/science.adq2178. PMID: 38662826; PMCID:PMC7616230.
2023
Quantitative 99mTc-albumin colloid nasal mucociliary clearance as an outcome in primary ciliary dyskinesia.
Authors: Marthin JK, Nielsen KG, Mortensen J.
ERJ Open Res. 2023 Sep 11;9(5):00345-2023. doi: 10.1183/23120541.00345-2023. eCollection 2023 Sep.
PMID: 37701364 Free PMC article.
Safety and efficacy of the epithelial sodium channel blocker idrevloride in people with primary ciliary dyskinesia (CLEAN-PCD): a
multinational, phase 2,randomised, double-blind, placebo-controlled crossover trial.
Authors: Ringshausen FC, Shapiro AJ, Nielsen KG, Mazurek H, Pifferi M, Donn KH, van der Eerden MM, Loebinger MR, Zariwala MA, Leigh
MW, Knowles MR, Ferkol TW; CLEAN-PCD investigators and study team. Lancet Respir Med. 2024 Jan;12(1):21-33. doi: 10.1016/S2213-2600(23)00226-6. Epub 2023 Aug 31.
PMID: 37660715 Clinical Trial.
Pulmonary radioaerosol mucociliary clearance assessment: searching for genotype-specific differences and potential as an outcome
measure in primary ciliary dyskinesia.
Authors: Marthin JK, Holgersen MG, Nielsen KG, Mortensen J.
ERJ Open Res. 2023 Dec 4;9(6):00685-2023. doi: 10.1183/23120541.00685-2023. eCollection 2023 Nov.
PMID: 38076675 Free PMC article.
Nasal nitric oxide measurement in children for the diagnosis of primary ciliary dyskinesia: European Respiratory Society technical standard.
Authors: Beydon N, Kouis P, Marthin JK, Latzin P, Colas M, Davis SD, Haarman E, Harris AL, Hogg C, Kilbride E, Kuehni CE, Marangu D, Nielsen KG, Pendergrast C, Robinson P, Rumman N, Rutter M, Walker WT, Ferkol T, Lucas JS. Eur Respir J. 2023 Apr 20;61(4):2202031. doi: 10.1183/13993003.02031-2022. Print 2023 Apr. PMID: 36822632
Towards the international interoperability of clinical research networks for rare diseases: recommendations from the IRDiRC Task
Force.
Authors: Nabbout R, Zanello G, Baker D, Black L, Brambilla I, Buske OJ, Conklin LS, Davies EH, Julkowska D, Kim Y, Klopstock T, Nakamura
H, Nielsen KG, Pariser AR, Pastor JC, Scarpa M, Smith M, Taruscio D, Groft S.
Orphanet J Rare Dis. 2023 May 9;18(1):109. doi: 10.1186/s13023-023-02650-4.
PMID: 37161573 Free PMC article.
Turning lung clearance index on its head. Reference data for SF6 multiple-breath washout derived ventilation distribution efficiency.
Authors: Sandvik RM, Lindblad A, Robinson PD, Nielsen KG, Gustafsson P. J Appl Physiol (1985). 2023 Feb 1;134(2):316-327. doi: 10.1152/japplphysiol.00541.2022. Epub 2022 Dec 22.
PMID: 36548514
Primary ciliary dyskinesia.
Authors: Raidt J, Loges NT, Olbrich H, Wallmeier J, Pennekamp P, Omran H. Presse Med. 2023 Jul 27;52(3):104171. doi: 10.1016/j.lpm.2023.104171. Online ahead of print. PMID: 37516247
Mucociliary Wnt signaling promotes cilia biogenesis and beating.
Authors: Seidl C, Da Silva F, Zhang K, Wohlgemuth K, Omran H, Niehrs C. Nat Commun. 2023 Mar 6;14(1):1259. doi: 10.1038/s41467-023-36743-2. PMID: 36878953 Free PMC article.
Crosstalk between cilia and autophagy: implication for human diseases.
Authors: Morleo M, Vieira HLA, Pennekamp P, Palma A, Bento-Lopes L, Omran H, Lopes SS, Barral DC, Franco B. Autophagy. 2023 Jan;19(1):24-43. doi: 10.1080/15548627.2022.2067383. Epub 2022 May 25. PMID: 35613303 Free PMC article. Review.
Pathogenic variants in CLXN encoding the outer dynein arm docking-associated calcium-binding protein calaxin cause primary ciliary dyskinesia.
Authors: Hjeij R, Aprea I, Poeta M, Nöthe-5. Menchen T, Bracht D, Raidt J, Honecker BI, Dougherty GW, Olbrich H, Schwartz O, Keller U, Nüsse H, Diderich KEM, Vogelberg C, Santamaria F, Omran H. Genet Med. 2023 May;25(5):100798. doi: 10.1016/j.gim.2023.100798. Epub 2023 Jan 31. PMID: 36727596
Molecular defects in primary ciliary dyskinesia are associated with agenesis of the frontal and sphenoid paranasal sinuses and chronic rhinosinusitis.
Authors: Schramm A, Raidt J, Gross A, Böhmer M, Beule AG, Omran H. Front Mol Biosci. 2023 Oct 3;10:1258374. doi: 10.3389/fmolb.2023.1258374. eCollection 2023. PMID: 37860582 Free PMC article.
Mapping the Most Common Founder Variant in RSPH9 That Causes Primary Ciliary Dyskinesia in Multiple Consanguineous Families of Bedouin Arabs. Al-Mutairi DA, Alsabah BH, Pennekamp P, Omran H. J Clin Med. 2023 Oct 13;12(20):6505. doi: 10.3390/jcm12206505. PMID: 37892643 Free PMC article.
The disease-specific clinical trial network for primary ciliary dyskinesia: PCD-CTN
2022
Chronic airway disease in primary ciliary dyskinesia-spiced with geno-phenotype associations
Authors: Nielsen KG, Holgersen MG, Crowley S, Marthin JK.. Am J Med Genet C Semin Med Genet. 2022 Mar 29. doi: 10.1002/ajmg.c.31967. Epub ahead of print. PMID: 35352480.
The disease-specific clinical trial network for primary ciliary dyskinesia: PCD-CTN
Authors: Johanna Raidt, Bernard Maitre, Petra Pennekamp, Josje Altenburg, Pinelopi Anagnostopoulou, Miguel Armengot, Lizan D Bloemsma, Mieke Boon, Melissa Borrelli, Folke Brinkmann, Siobhan B Carr, Mary P Carroll, Silvia Castillo-Corullón, André Coste, Renato Cutrera, Eleonora Dehlink, Damien M S Destouches, Maria E Di Cicco, Lucy Dixon, Nagehan Emiralioglu19, Ela Erdem Eralp 20, Eric G Haarman 21, Claire Hogg, Bulent Karadag, Helene E Kobbernagel 22, Natalie Lorent, Marcus A Mall, June K Marthin 22, Vendula Martinu 26, Manjith Narayanan, Ugur Ozcelik, Daniel Peckham, Massimo Pifferi, Petr Pohunek 26, Eva Polverino 29, Simon Range, Felix C Ringshausen, Evie Robson, Jobst Roehmel 23, Sandra Rovira-Amigo, Francesca Santamaria, Anne Schlegtendal, Zsolt Szépfalusi 15, Petra Tempels, Guillaume Thouvenin, Nicola Ullmann, Woolf T Walker, Martin Wetzke, Panayiotis Yiallouros, Heymut Omran, Kim G Nielsen. 2022 Aug 15;8(3):00139-2022.doi: 10.1183/23120541.00139-2022. eCollection 2022 Jul
Primary ciliary dyskinesia in Volendam: Diagnostic and phenotypic features in patients with a CCDC114 mutation.
Authors: Renate Kos, Joël Israëls, Christine D L van Gogh, Josje Altenburg, Sandra Diepenhorst, Tamara Paff, Elles M J Boon, Dimitra Micha, Gerard Pals, Anne H Neerincx, Anke H Maitland-van der Zee, Eric G Haarman, Amsterdam Mucociliary Clearance Disease (AMCD) Research Group. Am J Med Genet C Semin Med Genet. 2022 Mar;190(1):89-101. doi: 10.1002/ajmg.c.31968. Epub 2022 Mar
Crosstalk between cilia and autophagy: implication for human diseases.
Authors: Manuela Morleo, Helena L A Vieira, Petra Pennekamp, Alessandro Palma, Liliana Bento-Lopes, Heymut Omran, Susana S Lopes, Duarte C Barral, Brunella Franco. Autophagy. 2022 May 25;1-20.doi: 10.1080/15548627.2022.2067383. Online ahead of print.
Comparison of the Lung Clearance Index in Preschool Children With Primary Ciliary Dyskinesia and Cystic Fibrosis
Authors: Roehmel JF, Doerfler FJ, Koerner-Rettberg C, Brinkmann F, Schlegtendal A, Wetzke M, Rudolf I, Helms S, Große-Onnebrink J, Yu Y, Nuesslein T, Wojsyk-Banaszak I, Becker S, Eickmeier O, Sommerburg O, Omran H, Stahl M, Mall MA.. Chest. 2022 Mar 7:S0012-3692(22)00421-4. doi: 10.1016/j.chest.2022.02.052. Epub ahead of print. PMID: 35271842.
Limitations of Nasal Nitric Oxide Measurement for Diagnosis of Primary Ciliary Dyskinesia with Normal Ultrastructure
Authors: Raidt J, Krenz H, Tebbe J, Große-Onnebrink J, Olbrich H, Loges NT, Biebach L, Schmalstieg C, Keßler C, Wallmeier J, Dworniczak B, Pennekamp P, Dugas M, Werner C, Omran H.. Ann Am Thorac Soc. 2022 Feb 24. doi: 10.1513/AnnalsATS.202106-728OC. Epub ahead of print. PMID: 35202559.
2021
International BEAT-PCD consensus statement for infection prevention and control for primary ciliary dyskinesia in collaboration with ERN-LUNG PCD Core Network and patient representatives.
Authors: June K Marthin, Jane S Lucas, Mieke Boon, Carmen Casaulta, Suzanne Crowley, Damien M S Destouches, Ernst Eber, Amparo Escribano, Eric Haarman, Claire Hogg, Bernard Maitre, Gemma Marsh, Vendula Martinu, Antonio Moreno-Galdó, Huda Mussaffi, Heymut Omran, Petr Pohunek, Bernhard Rindlisbacher, Phil Robinson, Deborah Snijders, Woolf T Walker, Panayiotis Yiallouros, Helle Krogh Johansen, Kim G Nielsen. ERJ Open Res. 2021 Aug 2;7(3):00301-2021.doi: 10.1183/23120541.00301-2021. eCollection 2021 Jul.
Current and Future Treatments in Primary Ciliary Dyskinesia
Authors: Paff T, Omran H, Nielsen KG, Haarman EG.. Int J Mol Sci. 2021 Sep 11;22(18):9834. doi: 10.3390/ijms22189834. PMID: 34575997; PMCID: PMC8470068.
Lung clearance index predicts pulmonary exacerbations in individuals with primary ciliary dyskinesia: a multicentre cohort study.
Authors: Singer F, Schlegtendal A, Nyilas S, Vermeulen F, Boon M, Koerner-Rettberg C.
Longitudinal Lung Volume Changes by Ultrastructure and Genotype in Primary Ciliary Dyskinesia.
Authors: Pifferi M, Bush A, Mulé G, Gracci S, Fonnesu R, Michelucci A, Cangiotti A, Caligo MA, Miccoli M, Boner AL, Peroni D. Ann Am Thorac Soc. 2021 Jun;18(6):963-970.doi: 10.1513/AnnalsATS.202007-816OC.
2020
Access to medicines for rare diseases: beating the drum for primary ciliary dyskinesia.
Authors: Crowley S, Azevedo I, Boon M, Bush A, Eber E, Haarman E, Karadag B, Kötz K, Leigh M, Moreno-Galdó A, Mussaffi H, Nielsen KG, Omran H, Papon JF, Pohunek P, Priftis K, Rindlisbacher B, Santamaria F, Valiulis A, Witt M, Yiallouros P, Zivkovic Z, Kuehni CE, Lucas JS.
Clinical Features and Management of children with primary ciliary dyskinesia in England
Authors: Rubbo B, Best S, Hirst RA, Shoemark A, Goggin P, Carr SB, Chetcuti P, Hogg C, Kenia P, Lucas JS, Moya E, Narayanan M, O’Callaghan C, Williamson M, Walker WT; English National Children’s PCD Management Service. Arch Dis Child. 2020 Aug;105(8):724-729. doi: 10.1136/archdischild-2019-317687. Epub 2020 Mar 10. PMID: 32156696.
Efficacy and safety of azithromycin maintenance therapy in primary ciliary dyskinesia (BESTCILIA): a multicentre, double-blind, randomised, placebo-controlled phase 3 trial
Authors: Kobbernagel HE, Buchvald FF, Haarman EG, Casaulta C, Collins SA, Hogg C, Kuehni CE, Lucas JS, Moser CE, Quittner AL, Raidt J, Rosthøj S, Sørensen AL, Thomsen K, Werner C, Omran H, Nielsen KG.
Registries and collaborative studies for primary ciliary dyskinesia in Europe.
Authors: Ardura-Garcia C, Goutaki M, Carr SB, Crowley S, Halbeisen FS, Nielsen KG, Pennekamp P, Raidt J, Thouvenin G, Yiallouros PK, Omran H, Kuehni CE
2019
Primary ciliary dyskinesia in the genomics age.
Authors: Lucas JS, Davis SD, Omran H, Shoemark A. Lancet Respir Med. 2020 Feb;8(2):202-216. doi: 10.1016/S2213-2600(19)30374-1. Epub 2019 Oct 14.PMID: 31624012 Review.
One-year evolution and variability in multiple-breath washout indices in children and young adults with primary ciliary dyskinesia
Authors: Kobbernagel HE, Green K, Ring AM, Buchvald FF, Rosthøj S, Gustafsson PM, Nielsen KG. Eur Clin Respir J. 2019 Mar 26;6(1):1591841. doi: 10.1080/20018525.2019.1591841. PMID: 30949311; PMCID: PMC6442098.
Time trends in diagnostic testing for primary ciliary dyskinesia in Europe.
Authors: Halbeisen FS, Shoemark A, Barbato A, Boon M, Carr S, Crowley S, Hirst R, Karadag B, Koerner-Rettberg C, Loebinger MR, Lucas JS, Maitre B, Mazurek H, Özçelik U, Martinů V, Schwerk N, Thouvenin G, Tschanz SA, Yiallouros P, Goutaki M, Kuehni CE.
The Controversies and Difficulties of Diagnosing Primary Ciliary Dyskinesia (PCD).
Authors: Shoemark A, Rubbo B, Haarman E, Hirst RA, Hogg C, Jackson CL, Nielsen KN, Papon JF, Robinson P, Walker WT, Lucas JS.
SPEF2– and HYDIN-mutant Cilia Lack the Central Pair Associated Protein SPEF2 Aiding PCD Diagnostics.
Authors: Cindrić S, Dougherty GW, Olbrich H, Hjeij R, Loges NT, Amirav I, Philipsen MC, Marthin JK, Nielsen KG, Sutharsan S, Raidt J, Werner C, Pennekamp P, Dworniczak B, Omran H.
Pulmonary exacerbations in patients with primary ciliary dyskinesia: an expert consensus definition for use in clinical trials.
Authors: Lucas JS, Gahleitner F, Amorim A, Boon M, Brown P, Constant C, Cook S, Crowley S, Destouches DMS, Eber E, Mussaffi H, Haarman E, Harris A, Koerner-Rettberg C, Kuehni CE, Latzin P, Loebinger MR, Lorent N, Maitre B, Moreno-Galdó A, Nielsen KG, Özçelik U, Philipsen LKD, Pohunek P, Polverino E, Rademacher J, Robinson P, Snijders D, Yiallouros P, Carr SB.
De Novo Mutations in FOXJ1 Result in a Motile Ciliopathy with Hydrocephalus and Randomization of Left/Right Body Asymmetry.
Authors: Wallmeier J, Frank D, Shoemark A, Nöthe-Menchen T, Cindric S, Olbrich H, Loges NT, Aprea I, Dougherty GW, Pennekamp P, Kaiser T, Mitchison HM, Hogg C, Carr SB, Zariwala MA, Ferkol T, Leigh MW, Davis SD, Atkinson J, Dutcher SK, Knowles MR, Thiele H, Altmüller J, Krenz H, Wöste M, Brentrup A, Ahrens F, Vogelberg C, Morris-Rosendahl DJ, Omran H.
2025
Detection and evaluation of drug-associated pulmonary arterial hypertension.
Authors: Jambon-Barbara C, Hlavaty A, Grynblat J, Antigny F, Chaumais MC, Humbert M, Cracowski JL, Montani D, Khouri C. Am J Respir Crit Care Med 2025; 211: 2158–68.
Cardiopulmonary hemodynamic determinants of long-term survival in sickle cell disease: insights from the ETENDARD study.
Authors: Savale L, D’humières T, Boucly A, Habibi A, Lionnet F, Loko G, Inamo J, De Luna G, Al Khaf S, Chantalat-Auger C, Maitre B, Jaïs X, Montani D, Sitbon O, Parent F, Simonneau G, Galacteros F, Audureau E, Bartolucci P, Humbert M Am J Respir Crit Care Med 2025; 211: 2096-104.
Sotatercept for pulmonary arterial hypertension within the first year after diagnosis
Authors: McLaughlin VV, Hoeper MM, Badesch DB, Ghofrani HA, Gibbs JSR, Gomberg-Maitland M, Preston IR, Souza R, Waxman AB, Kopeć G, Meyer G, Olsson KM, Fu W, Shi Y, Miller B, Kim SS, Mackenzie HS, Brambatti M, Patel MJ, Koglin J, Cornell AG, Humbert M.. N Engl J Med 2025; 393:1599-611.
Overlapping pulmonary hypertension phenotypes in smokers: a call to action.
Authors: Syed M, Humbert M, Kovacs G, Sahay S. Lancet Respir Med 2025; 13:867-70.
Association between anticoagulant therapy and survival in pulmonary arterial hypertension: a registry report and updated meta-analysis.
Authors: Bertoletti L, Escal J, Boucly A, Turquier S, Jevnikar M, Lamblin N, Bezzeghoud S, Chapelle C, Delavenne X, Savale L, Humbert M, Montani D, Sitbon O, Jais X. J Am Coll Cardiol 2025; 86:982-95.
Pericardial effusion in sotatercept phase 3 trials: insights from STELLAR and ZENITH. Authors: Hoeper MM, Preston IR, Gomberg-Maitland M, Waxman AB, Shi Y, Lin J, Mackenzie HS, Loureiro MJ, McLaughlin VV, Humbert M. Eur Respir J 2025; 66:2500768.
Clustering pulmonary hypertension patients using the plasma proteome.
Authors: Boucly A, Song S, Keles M, Wang D, Howard LS, Humbert M, Sitbon O, Lawrie A, Thompson AAR, Frank P, Kivimaki M, Rhodes CJ, Wilkins MR. Am J Respir Crit Care Med 2025; 211:1492-503.
Drugs targeting novel pathways in pulmonary arterial hypertension.
Authors: Sitbon O, Boucly A, Weatherald J, Antigny F, Guignabert C, Jevnikar M, Jaïs X, Price LC, Wilkins MR, Zamanian RT, Savale L, Montani D, Humbert M. Eur Respir J 2025; 66:2401830.
Sotatercept in patients with pulmonary arterial hypertension at high risk for death.
Authors: Humbert M, McLaughlin VV, Badesch DB, Ghofrani HA, Gibbs JSR, Gomberg-Maitland M, Preston IR, Souza R, Waxman AB, Moles VM, Savale L, Vizza CD, Rosenkranz S, Shi Y, Miller B, Mackenzie HS, Kim SS, Loureiro MJ, Patel MJ, Koglin J, Cornell AG, Hoeper MM. N Engl J Med 2025; 392:1987-2000.
Consistent safety and efficacy of sotatercept for pulmonary arterial hypertension in BMPR2 mutation carriers and noncarriers: a planned analysis of Phase 2, double-blind, placebo-controlled clinical trial (PULSAR).
Authors: Montani D, McLaughlin VV, Gibbs JSR, Gomberg-Maitland M, Hoeper MM, Preston IR, Souza R, Waxman AB, Escribano Subias P, Feldman J, Meyer GM, Olsson KM, Coulet F, Manimaran S, Zhao Y, Lau A, de Oliveira Pena J, Badesch DB, Humbert M. Am J Respir Crit Care Med 2025; 211:1028-37.
Future treatment paradigms in pulmonary arterial hypertension: a personal view from physicians, health authorities, and patients.
Authors: Rahaghi FF, Humbert M, Hoeper MM, White RJ, Frantz RP, Hassoun PM, Hemnes AR, Kawut SM, McLaughlin VV, Meszaros G, Mol PGM, Nathan SD, Psotka MA, Rahaghi FN, Sitbon O, Stockbridge N, Weatherald J, Zannad F, Sahay SLancet Respir Med 2025; 13:364-70.
PFMG2025-integrating genomic medicine into the national healthcare system in France.
Authors: PFMG2025 contributors. Lancet Reg Health Eur 2025; 50:101183.
Initial therapy in patients with pulmonary arterial hypertension and cardiovascular comorbidities.
Authors: Boucly A, Montani D, Bauer F, Artaud-Macari E, Bergot E, Boissin C, Chaouat A, Cottin V, Dauphin C, Degano B, De Groote P, du Roure C, Favrolt N, Horeau-Langlard D, Jaïs X, Jevnikar M, Lacoste-Palasset T, Picard F, Prévôt G, Reynaud-Gaubert M, Roche A, Turquier S, Humbert M, Sitbon O, Savale L. Eur Respir J 2025; 65:2400895.
Efficacy and safety of the activin signalling inhibitor, sotatercept, in a pooled analysis of PULSAR and STELLAR studies.
Authors: Hoeper MM, Gomberg-Maitland M, Badesch DB, Gibbs JSR, Grünig E, Kopeć G, McLaughlin VV, Meyer G, Olsson KM, Preston IR, Rosenkranz S, Souza R, Waxman AB, Perchenet L, Strait J, Xing A, Manimaran S, Wang X, Miller B, Cornell AG, de Oliveira Pena J, Ghofrani HA, Humbert M Eur Respir J 2025; 65:2401424
Seralutinib for the Treatment of Pulmonary Arterial Hypertension in Adults: TORREY Open-Label Extension Study.
Authors: Sitbon, O. et al. Lancet Respir Med. 10/2025. doi: 10.1007/s12325-025-03297-2.
Pulmonary arterial hypertension in adults with Still’s disease: another pulmonary manifestation associated with HLA-DRB1*15.
Authors: Boucly, A. Ann Rheum Dis. 09/2025. doi: 10.1016/j.ard.2025.04.016.
Highlights for the Heart Failure Cardiologist from the Seventh World Symposium on Pulmonary Hypertension: Are We Out of the Woods Yet?Authors: Cascino T. J cardiac Fail. 01/2025. doi: 10.1016/j.cardfail.2024.09.009.
Right ventricular reserve in cardiopulmonary disease: A simultaneous hemodynamic and three-dimensional echocardiographic study.
Authors: Baratto C. J Heart Lung Transplant. 06/2025. doi: 10.1016/j.healun.2024.12.022.
ZENITH Trial Investigators. Sotatercept in Patients with Pulmonary Arterial Hypertension at High Risk for Death.
Authors: Humbert M. New Engl J Med. 05/2025. doi: 10.1056/NEJMoa2415160.
Approach to Lung Transplantation in Pulmonary Arterial Hypertension: A Delphi Consensus on Behalf of the Transplant Task Force of the Pulmonary Vascular Research Institute.
Authors: Kolaitis N. Pulm Circ. 04/2025. doi: 10.1002/pul2.70088.
Echocardiographic practices in pulmonary hypertension: survey results and literature review.
Authors: Lancellotti P. Acta Cardiologica. 06/2025. doi: 10.1080/00015385.2025.2518645.
Delphi Study Contributing Group. Key elements of follow-up care after acute pulmonary embolism focusing on long-term sequelae: a Delphi study among European experts.
Authors: Mali RMA. Eur Heart J Qual Care Clin Outcomes. 11/2025. doi: 10.1093/ehjqcco/qcaf053.
Determinants of Right Heart Hemodynamic Derangement in Patients With and Without Tricuspid Regurgitation.
Authors: Caravita S. Circ Heart Fail. 08/2025. doi: 10.1161/CIRCHEARTFAILURE.125.012813.
TORREY Study Investigators. Seralutinib for the Treatment of Pulmonary Arterial Hypertension in Adults: TORREY Open-Label Extension Study.
Authors: Sitbon O. Adv Ther. 08/2025. doi: 10.1016/j.healun.2025.04.015.
HYPERION Trial Investigators. Sotatercept for Pulmonary Arterial Hypertension within the First Year after Diagnosis.
Authors: McLaughlin VV. N Engl J Med. 10/2025. doi: 10.1056/NEJMoa2508170.
International Society for Heart and Lung Transplantation (ISHLT) Consensus Statement on Risk Stratification in Pulmonary Arterial Hypertension.
Authors: Sahay S. J Heart Lung Transplant. 11/2025. doi: 10.1016/j.healun.2025.
Evolving paradigms in pulmonary hypertension: Highlights of the 6th French Pulmonary Hypertension Network Meeting.
Authors: Boucly A. Respir Med Res. 11/2025. doi: 10.1016/j.resmer.2025.101215.
What glitters is not always gold: image focus of an intimal sarcoma of the right pulmonary artery.
Authors: Christiansen Gozzer E. Acta Cardiol.. 12/2025. doi: 10.1080/00015385.2025.2576452.
Diagnostic Performance of Passive Leg-Raise and Low-Workload Exercise to Diagnose Heart Failure With Preserved Ejection Fraction.
Authors: Baratto C. J Card Fail. 11/2025. doi: 10.1016/j.cardfail.2025.09.006.
A long-term follow-up study of sotatercept for treatment of pulmonary arterial hypertension: interim results of SOTERIA.
Authors: Preston IR. Eur Respir J. 07/2025. doi: 10.1183/13993003.01435-2024.
Clinical characteristics and outcomes of systemic sclerosis–associated pulmonary arterial hypertension in real-world registries: an individual patient data meta-analysis.
Authors: Grünig E. Eur Respir J. 12/2025. doi: 10.1183/13993003.01234-2025
Detection of structural pulmonary changes with real-time high-fidelity analysis of expiratory CO₂.
Authors: Verbraecken J. ERJ Open Res. 10/2025. doi: 10.1183/23120541.00987-2025.
Characterizing SV40-hTERT Immortalized Human Lung Microvascular Endothelial Cells as Model System for Mechanical Stretch-Induced Lung Injury.
Authors: Hochreiter B. Int J Mol Sci .. 01/2025. doi: doi: 10.3390/ijms26020683.. PMID: 39859396
3D Reconstruction of Pulmonary Vascular Lesions in Chronic Thromboembolic Pulmonary Hypertension.
Authors: Ryota Kakizaki . Circ Cardiovasc Imaging. 08/2025. doi: 10.1161/CIRCIMAGING.124.017935.. PMID: 39996323
Long-Term Treatment With Subcutaneous Treprostinil in Patients With Severe Inoperable Chronic Thromboembolic Pulmonary Hypertension in the Multimodal Therapy Era (Data From CTREPH Study Open Label Extension).
Authors: Pavel Jansa. Pulm Circ .. 04/2025. doi: 10.1002/pul2.70080. eCollection 2025 Apr.. PMID: 40291433
A reason to celebrate: multimodal treatments and recent improvements in the long-term outcomes of patients with chronic thromboembolic pulmonary hypertension.
Authors: Irene Lang. Eur Respir J .. 07/2025. doi: 10.1183/13993003.00562-2025.. PMID: 40659467
Long-term effects of medical treatment in patients with chronic thromboembolic pulmonary hypertension.
Authors: Marion Delcroix. ERJ Open Res .. 12/2025. doi: 10.1183/23120541.00769-2025. PMID: 41367646
Risk in acute pulmonary embolism: how can we estimate it RIGHT?
Authors: Irene Lang. Eur Heart J .. 12/2025. doi: 10.1093/eurheartj/ehaf393. PMID: 40748623
Timing of mechanical treatments for acute pulmonary embolism: earlier is better.
Authors: Irene Lang. EuroIntervention. 05/2025. doi: 10.4244/EIJ-E-25-00016. PMID: 40325987
Optimal approach to performing and reporting computed tomography angiography for suspected acute pulmonary embolism: a clinical consensus statement of the ESC Working Group on Pulmonary Circulation & Right Ventricular Function, the Fleischner Society, the Association for Acute CardioVascular Care (ACVC) and the European Association of Cardiovascular Imaging (EACVI) of the ESC, endorsed by European Respiratory Society (ERS), Asian Society of Thoracic Radiology (ASTR), European Society of Thoracic Imaging (ESTI), and Society of Thoracic Radiology (STR).
Authors: F A Klok. Eur Heart J Cardiovasc Imaging .. 06/2025. doi: 10.1093/ehjci/jeaf050. PMID: 40457988
Chronic Thromboembolic Pulmonary Hypertension and Cancer: a true relationship, but one likely beyond coagulation.
Authors: Christian Gerges. J Heart Lung Transplant .. 03/2025. doi: 10.1016/j.healun.2024.11.025. PMID: 39586483
Rationale for and approach to establishing a multidisciplinary acute pulmonary embolism expert care team.
Authors: F A Klok. Eur Heart J Acute Cardiovasc Care .. 10/2025. doi: 10.1093/ehjacc/zuaf141. PMID: 41159543
The Prognostic Implication of Late Gadolinium Enhancement Quantification and Syncope in Hypertrophic Cardiomyopathy.
Authors: Christopher Mann. J Clin Med .. 03/2025. doi: 10.3390/jcm14051781. PMID: 40095924
Interventional strategies to unload the right ventricle: a systematic review.
Authors: Leila Alajbegovic. Expert Rev Respir Med .. 07/2025. doi: 10.1080/17476348.2025.2495164. PMID: 40259525
Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension: Results of an International Multicenter Prospective Registry.
Authors: Irene M Lang. J Am Coll Cardiol .. 06/2025. doi: 10.1016/j.jacc.2025.04.021. PMID: 40499982
Diagnosis of chronic thromboembolic pulmonary hypertension.
Authors: Xavier Jais. J Heart Lung Transplant .. 07/2025. doi: 10.1016/j.healun.2025.02.1688. PMID: 40653349
Management of chronic thromboembolic pulmonary hypertension.
Authors: Christoph B Wiedenroth . J Heart Lung Transplant . 07/2025. doi: 10.1016/j.healun.2025.02.1691. PMID: 40653350
Fibrinolytic Therapy for Thromboembolic Diseases: Approved Indications and Future Directions.
Authors: Sina Rashedi. J Am Coll Cardiol .. 10/2025. doi: 10.1016/j.jacc.2025.07.061. PMID: 41033744
Effects of macitentan and selexipag across prognostic age groups in patients with pulmonary arterial hypertension.
Authors: Richard Channick. JHLT open. 01/2025. doi: 10.1016/j.jhlto.2024.100197. PMID: 40144855
Outcomes in Patients Receiving Treatment for Pulmonary Arterial Hypertension Associated With Repaired Congenital Heart Disease.
Authors: Richard A Krasuski. JACC Adv .. 03/2025. doi: 10.1016/j.jacadv.2025.101626. PMID: 39999520
Development of a Novel, Pulmonary Endovascular Device to Treat Patients With Pulmonary Hypertension.
Authors: Karl Vollmers. Pulm Circ .. 07/2025. doi: 10.1002/pul2.70131. PMID: 40741160
Lung transplantation for chronic thromboembolic pulmonary hypertension (CTEPH): A treatment of last resort.
Authors: Edmund M Lau. J Heart Lung Transplant .10/2025. doi: 10.1016/j.healun.2025.10.006. PMID: 41106442
Pulmonary arterial stiffness as the main correlate of effective arterial elastance in pre- and post-capillary pulmonary hypertension.
Authors: Denis Chemla. Respir Med Res. 11/2025. doi: 10.1016/j.resmer.2025.101204. PMID: 41067037
Effect of Balloon Pulmonary Angioplasty and Riociguat on Right Ventricular Afterload and Function in CTEPH: Insights From the RACE Trial.
Authors: Christian Gerges. Circ Cardiovasc Interv .. 02/2025. doi: 10.1161/CIRCINTERVENTIONS.124.014785. PMID: 39965044
Obesity and Acute Pulmonary Embolism: Clinical Implications and Knowledge Gaps.
Authors: Marco Zuin. J Am Coll Cardiol .. 11/2025. doi: 10.1016/j.jacc.2025.09.1504. PMID: 41224406
2024
Pulmonary hypertension.
Mocumbi A, Humbert M, Saxena A, Jing ZC, Sliwa K, Thienemann F, Archer SL, Stewart S.Nat Rev Dis Primers. 2024 Jan 4;10(1):1. doi: 10.1038/s41572-023-00486-7.PMID: 38177157 Review.
Pulmonary hypertension associated with lung diseases.
Shlobin OA, Adir Y, Barbera JA, Cottin V, Harari S, Jutant EM, Pepke-Zaba J, Ghofrani HA, Channick R.Eur Respir J. 2024 Oct 31;64(4):2401200. doi: 10.1183/13993003.01200-2024. Print 2024 Oct.PMID: 39209469 Free PMC article. Review.
Phenotypes in pulmonary hypertension.
Weatherald J, Hemnes AR, Maron BA, Mielniczuk LM, Gerges C, Price LC, Hoeper MM, Humbert M.Eur Respir J. 2024 Sep 5;64(3):2301633. doi: 10.1183/13993003.01633-2023. Print 2024 Sep.PMID: 38964779 Review.
[Pulmonary Embolism].
Barca-Hernando M, García-Ortega A, Martínez-Meñaca A, Ramírez-Martín MP, Rivas-Guerrero A, Tenes A.Open Respir Arch. 2024 Jun 4;6(4):100342. doi: 10.1016/j.opresp.2024.100342. eCollection 2024 Oct-Dec.PMID: 39035608 Free PMC article. Review. Spanish.
Pathology and pathobiology of pulmonary hypertension: current insights and future directions.
Guignabert C, Aman J, Bonnet S, Dorfmüller P, Olschewski AJ, Pullamsetti S, Rabinovitch M, Schermuly RT, Humbert M, Stenmark KR.Eur Respir J. 2024 Oct 31;64(4):2401095. doi: 10.1183/13993003.01095-2024. Print 2024 Oct.PMID: 39209474 Free PMC article. Review.
Risk Stratification in Pulmonary Veno-Occlusive Disease.
Cruz-Utrilla A, Pérez-Olivares C, Luna-López R, Jiménez López-Guarch C, Bedate P, Martínez Meñaca A, López Meseguer M, Escribano-Subias P.Arch Bronconeumol. 2024 May;60(5):321-323. doi: 10.1016/j.arbres.2024.02.011. Epub 2024 Feb 24.PMID: 38461109 English, Spanish. No abstract available.
Expert management of congenital portosystemic shunts and their complications.
McLin VA, Franchi-Abella S, Brütsch T, Bahadori A, Casotti V, de Ville de Goyet J, Dumery G, Gonzales E, Guérin F, Hascoet S, Heaton N, Kuhlmann B, Lador F, Lambert V, Marra P, Plessier A, Quaglia A, Rougemont AL, Savale L, Sarma MS, Sitbon O, Superina RA, Uchida H, van Albada M, van der Doef HPJ, Vilgrain V, Wacker J, Zwaveling N, Debray D, Wildhaber BE.JHEP Rep. 2023 Oct 20;6(1):100933. doi: 10.1016/j.jhepr.2023.100933. eCollection 2024 Jan.PMID: 38234409 Free PMC article. Review.
Publisher Correction: Pulmonary hypertension.
Mocumbi A, Humbert M, Saxena A, Jing ZC, Sliwa K, Thienemann F, Archer SL, Stewart S.Nat Rev Dis Primers. 2024 Jan 17;10(1):5. doi: 10.1038/s41572-024-00493-2.PMID: 38233482 No abstract available.
[Highlights 57th SEPAR Congress].
Martínez Meñaca A, García Moyano M, Sánchez-Salcedo P, Cascón-Hernández J, Sante Diciolla N, Muñoz-Ezquerre M, Barbero Herranz E, Alonso Pérez T.Open Respir Arch. 2024 Sep 5;6(4):100360. doi: 10.1016/j.opresp.2024.100360. eCollection 2024 Oct-Dec.PMID: 39351171 Free PMC article. Review. Spanish.
Comparative Effectiveness of Sotatercept and Approved Add-On Pulmonary Arterial Hypertension Therapies: A Systematic Review and Network Meta-Analysis.
Pitre T, Desai K, Mah J, Zeraatkar D, Humbert M.Ann Am Thorac Soc. 2024 Aug;21(8):1194-1203. doi: 10.1513/AnnalsATS.202311-942OC.PMID: 38820258
Effect of sotatercept on circulating proteomics in pulmonary arterial hypertension.
Savale L, Tu L, Normand C, Boucly A, Sitbon O, Montani D, Olsson KM, Park DH, Fuge J, Kamp JC, Humbert M, Hoeper MM, Guignabert C.Eur Respir J. 2024 Oct 31;64(4):2401483. doi: 10.1183/13993003.01483-2024. Print 2024 Oct.PMID: 39227073 Free PMC article.
Role of Oxygen Starvation in Right Ventricular Decompensation and Failure in Pulmonary Arterial Hypertension.
Oknińska M, Zajda K, Zambrowska Z, Grzanka M, Paterek A, Mackiewicz U, Szczylik C, Kurzyna M, Piekiełko-Witkowska A, Torbicki A, Kieda C, Mączewski M.JACC Heart Fail. 2024 Feb;12(2):235-247. doi: 10.1016/j.jchf.2023.03.010. Epub 2023 May 3.PMID: 37140511 Review.
Pulmonary arterial wedge pressure in healthy subjects: a meta-analysis.
Zeder K, Avian A, Mak S, Giannakoulas G, Kawut SM, Maron BA, Humbert M, Olschewski H, Kovacs G.Eur Respir J. 2024 Aug 15;64(2):2400967. doi: 10.1183/13993003.00967-2024. Print 2024 Aug.PMID: 38964777 Free PMC article.
The Seventh World Symposium on Pulmonary Hypertension: our journey to Barcelona.
Humbert M, Galiè N, Rubin LJ, Simonneau G, McLaughlin VV.Eur Respir J. 2024 Oct 31;64(4):2401222. doi: 10.1183/13993003.01222-2024. Print 2024 Oct.PMID: 39209470 Free PMC article.
Primum non nocere, secundum cavere, tertium sanare: lessons on the management of pulmonary hypertension associated with COPD.
Kovacs G, Humbert M.Eur Respir J. 2024 Jun 6;63(6):2400765. doi: 10.1183/13993003.00765-2024. Print 2024 Jun.PMID: 38843938 No abstract available.
Pulmonary Hypertension in Connective Tissue Diseases Other than Systemic Sclerosis.
Budhram B, Weatherald J, Humbert M.Semin Respir Crit Care Med. 2024 Jun;45(3):419-434. doi: 10.1055/s-0044-1782217. Epub 2024 Mar 18.PMID: 38499196 Review.
Initial combination therapy with macitentan and tadalafil in patients with pulmonary arterial hypertension, with and without cardiac comorbidities.
McLaughlin VV, Sitbon O, Chin KM, Galiè N, Hoeper MM, Kiely DG, MacDonald G, Martin N, Mathai SC, Peacock A, Tawakol A, Torbicki A, Noordegraaf AV, Rosenkranz S.Eur J Heart Fail. 2024 Nov;26(11):2379-2391. doi: 10.1002/ejhf.3319. Epub 2024 Jul 5.PMID: 38966990 Free PMC article. Clinical Trial.
Pulmonary Hypertension: From Therapeutic Nihilism to Multiple Therapeutic Interventions.
McLaughlin VV, Humbert M.Circulation. 2024 Nov 19;150(21):1653-1655. doi: 10.1161/CIRCULATIONAHA.124.070104. Epub 2024 Nov 18.PMID: 39556653 Free article. No abstract available.
Exploring the patient perspective in pulmonary hypertension.
Ford HJ, Brunetti C, Ferrari P, Meszaros G, Moles VM, Skaara H, Torbicki A, Gibbs JSR.Eur Respir J. 2024 Oct 31;64(4):2401129. doi: 10.1183/13993003.01129-2024. Print 2024 Oct.PMID: 39209479 Free PMC article.
Pulmonary vascular phenotype identified in patients with GDF2 (BMP9) or BMP10 variants: an international multicentre study.
Grynblat J, Bogaard HJ, Eyries M, Meyrignac O, Savale L, Jaïs X, Ghigna MR, Celant L, Meijboom L, Houweling AC, Levy M, Antigny F, Chaouat A, Cottin V, Guignabert C, Coulet F, Sitbon O, Bonnet D, Humbert M, Montani D; and the French PH Network PULMOTENSION Investigators.Eur Respir J. 2024 Apr 4;63(4):2301634. doi: 10.1183/13993003.01634-2023. Print 2024 Apr.PMID: 38514094 Review.
Pulmonary hypertension in patients carrying FLNA loss-of-function variants.
Stourm L, Grynblat J, Savale L, Lacoste-Palasset T, Jaïs X, Coulet F, Levy M, Meyrignac O, Ghigna MR, Cottin V, Sitbon O, Bonnet D, Goupil F, Humbert M, Gagnadoux F, Montani D; French PH Network PULMOTENSION Investigators.Eur Respir J. 2024 Nov 7:2401132. doi: 10.1183/13993003.01132-2024. Online ahead of print.PMID: 39510553
Treatments for pulmonary arterial hypertension: navigating through a network of choices.
Pitre T, Weatherald J, Humbert M.Eur Heart J. 2024 Jun 1;45(21):1953-1955. doi: 10.1093/eurheartj/ehae106.PMID: 38442135 No abstract available.
Safety and efficacy of rodatristat ethyl for the treatment of pulmonary arterial hypertension (ELEVATE-2): a dose-ranging, randomised, multicentre, phase 2b trial.
Sitbon O, Skride A, Feldman J, Sahay S, Shlobin OA, McLaughlin V, Ghofrani HA, Langleben D, Parsley E, D’Souza G, Marmon T, Kamau-Kelley W, Jones R, Grewal R, Wring S, Palacios M, Naik H, Denning J, Lazarus HM, Humbert M.Lancet Respir Med. 2024 Nov;12(11):865-876. doi: 10.1016/S2213-2600(24)00226-1. Epub 2024 Sep 19.PMID: 39307144 Clinical Trial.
Simplified risk stratification based on cardiopulmonary exercise test: A Spanish two-center experience.
Martínez-Meñaca A, Cruz-Utrilla A, Mora-Cuesta VM, Luna-López R, Segura-de la Cal T, Flox-Camacho Á, Alonso-Lecue P, Escribano-Subias P, Cifrián-Martínez JM.Pulm Circ. 2024 Feb 27;14(1):e12342. doi: 10.1002/pul2.12342. eCollection 2024 Jan.PMID: 38414916 Free PMC article.
Chronic Thromboembolic Pulmonary Hypertension: When Thrombus Mandates Definite Diagnosis to Occur in the Operating Room.
Martín de Miguel I, López-Gude MJ, Martínez Meñaca A, Revilla Ostolaza Y, Arribas Ynsaurriaga F, Escribano Subías P.Circ Cardiovasc Imaging. 2024 Sep;17(9):e016543. doi: 10.1161/CIRCIMAGING.123.016543. Epub 2024 Jun 5.PMID: 38836344 No abstract available.
MERIT reloaded-what has changed in CTEPH management since 2017?
Torbicki A, Kurzyna M.Lancet Respir Med. 2024 Apr;12(4):263-265. doi: 10.1016/S2213-2600(24)00040-7.PMID: 38548405 No abstract available.
Development of a Nomogram for Genetic Risk of PAH.
Cruz-Utrilla A, Vargas-Ursúa F, Fernández-Villar A, Escribano-Subías P.Arch Bronconeumol. 2024 Oct 22:S0300-2896(24)00404-6. doi: 10.1016/j.arbres.2024.10.001. Online ahead of print.PMID: 39516168 English, Spanish. No abstract available.
Effect of Macitentan in Pulmonary Arterial Hypertension and the Relationship Between Echocardiography and cMRI Variables: REPAIR Echocardiography Sub-study Results.
Torbicki A, Channick R, Galiè N, Kiely DG, Moceri P, Peacock A, Swift AJ, Tawakol A, Vonk Noordegraaf A, Flores D, Martin N, Rosenkranz S.Cardiol Ther. 2024 Mar;13(1):173-190. doi: 10.1007/s40119-023-00345-2. Epub 2024 Jan 28.PMID: 38281309 Free PMC article.
Clinical and echocardiographic characterization of patients hospitalized for severe tricuspid valve regurgitation: a single tertiary-care center experience with 2-year follow-up.
Piasecki A, Tomaniak M, Gumiężna K, Kurzyna P, Bednarek A, Skulimowska J, Pedzich E, Kapłon-Cieślicka A, Rdzanek A, Ścisło P.Pol Arch Intern Med. 2024 Aug 8;134(7-8):16752. doi: 10.20452/pamw.16752. Epub 2024 May 15.PMID: 38752413 Free article.
Prognostic relevance of exercise pulmonary hypertension: results of the multicentre PEX-NET Clinical Research Collaboration.
Kovacs G, Humbert M, Avian A, Lewis GD, Ulrich S, Vonk Noordegraaf A, Souza R, Galiè N, Malhotra R, Saxer S, Grünig E, Egenlauf B, Ewert R, Heine A, Tedford RJ, Houston BA, Kasperowicz K, Kurzyna M, Rosenkranz S, Herkenrath S, Barbera JA, Blanco I, Oliveira RKF, Andersen M, Savale L, Systrom D, Maron BA, Tello K, Condliffe R, Mak S, Baratto C, Hsu S, D’Alto M, McCabe C, Herve P, Olschewski H; PEX-NET (Pulmonary Haemodynamics during Exercise Network) ERS Clinical Research Collaboration.Eur Respir J. 2024 Nov 27;64(6):2400698. doi: 10.1183/13993003.00698-2024. Print 2024 Dec.PMID: 39603672 Free PMC article.
Transcatheter correction of a rare combined anomalous pulmonary and systemic venous return in an adult.
Albenque G, Batteux C, Hascoët S.Eur Heart J. 2024 Dec 16;45(47):5100. doi: 10.1093/eurheartj/ehae732.PMID: 39471415 No abstract available.
Corrigendum to “Expert management of congenital portosystemic shunts and their complications” [JHEP Reports 6 (2024)].
McLin VA, Franchi-Abella S, Brütsch T, Bahadori A, Casotti V, de Ville de Goyet J, Dumery G, Gonzales E, Guérin F, Hascoet S, Heaton N, Kuhlmann B, Lador F, Lambert V, Marra P, Plessier DA, Quaglia A, Rougemont AL, Savale L, Sarma MS, Sitbon O, Superina RA, Uchida H, van Albada M, Johannes van der Doef HP, Vilgrain V, Wacker J, Zwaveling-Soonawala N, Debray D, Wildhaber BE.JHEP Rep. 2024 Jan 30;6(3):101024. doi: 10.1016/j.jhepr.2024.101024. eCollection 2024 Mar.PMID: 38481635 Free PMC article.
Epidemiology of supplemental oxygen in patients with pulmonary hypertension.
Torres-Castro R, Hinojosa W, Martínez-Meñaca A, Sala Llinas E, Jiménez Arjona J, Rueda Soriano J, Aurtenetxe A, Barberà JA, Escribano-Subías P, Blanco I; REHAP Investigators.Respirology. 2025 Jan;30(1):70-79. doi: 10.1111/resp.14821. Epub 2024 Aug 29.PMID: 39210654 Free article.
Quality of Life and the Cardiopulmonary Exercise Test in Pulmonary Arterial Hypertension Patients.
Martínez-Meñaca A, Mora-Cuesta VM, Iturbe-Fernández D, Sáinz-Ezquerra Belmonte B, Fernández-Cavia G, Gallardo-Ruiz MJ.Arch Bronconeumol. 2024 Apr;60(4):253-255. doi: 10.1016/j.arbres.2024.01.016. Epub 2024 Feb 13.PMID: 38402048 English, Spanish. No abstract available.
The Initial Experience of Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension in Latvia.
Krigere A, Kalejs VR, Kaulins R, Rudzitis A, Bondare L, Sablinskis M, Lejnieks A, Kigitovica D, Kurzyna M, Skride A.Medicina (Kaunas). 2024 Mar 30;60(4):568. doi: 10.3390/medicina60040568.PMID: 38674214 Free PMC article.
Comment on the article titled “Impact of sex on outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension” published by J. Chan et al.
Cruz-Utrilla A, López-Gude MJ, Velázquez M, Arribas-Ynsaurriaga F, Escribano-Subias P.J Heart Lung Transplant. 2024 Apr;43(4):689-690. doi: 10.1016/j.healun.2023.12.008. Epub 2023 Dec 21.PMID: 38141892 No abstract available.
Safety and Outcomes of Inferior Vena Cava Filter Placement in Oncology Patients: A Single-Centre Experience.
Kurzyna P, Banaszkiewicz M, Florczyk M, Kępski J, Piłka M, Kędzierski P, Mańczak R, Szwed P, Kasperowicz K, Wrona K, Doroszewski G, Torbicki A, Kurzyna M, Szmit S, Darocha S.Cancers (Basel). 2024 Apr 19;16(8):1562. doi: 10.3390/cancers16081562.PMID: 38672644 Free PMC article.
Anxiety and Depression in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension after the Removal of COVID-19 Pandemic Restrictions.
Wieteska-Miłek M, Witowicz A, Szmit S, Florczyk M, Peller M, Dzienisiewicz M, Kurzyna M.J Clin Med. 2024 Jun 17;13(12):3532. doi: 10.3390/jcm13123532.PMID: 38930062 Free PMC article.
Prognostic relevance of exercise pulmonary hypertension: results of the multicentre PEX-NET Clinical Research Collaboration.
Authors: Kovacs G, Humbert M, Avian A, Lewis GD, Ulrich S, Vonk Noordegraaf A, Souza R, Galiè N, Malhotra R, Saxer S, Grünig E, Egenlauf B, Ewert R, Heine A, Tedford RJ, Houston BA, Kasperowicz K, Kurzyna M, Rosenkranz S, Herkenrath S, Barbera JA, Blanco I, Oliveira RKF, Andersen M, Savale L, Systrom D, Maron BA, Tello K, Condliffe R, Mak S, Baratto C, Hsu S, D’Alto M, McCabe C, Herve P, Olschewski H; PEX-NET (Pulmonary Haemodynamics during Exercise Network) ERS Clinical Research Collaboration. Eur Respir J. 2024 Nov 27;64(6):2400698. doi: 10.1183/13993003.00698-2024. Print 2024 Dec. PMID: 39603672 Free PMC article.
Changes in REVEAL Lite 2 risk status are associated with long-term outcomes in patients with pulmonary arterial hypertension: A post-hoc analysis of the GRIPHON study.
Authors: Benza RL, Chin KM, Gaine S, Galiè N, Hoeper MM, Lang IM, McLaughlin VV, Sitbon O, Doad G, Yen J, Tang X, Tapson V. J Heart Lung Transplant. 2024 Dec;43(12):1998-2007. doi: 10.1016/j.healun.2024.08.019. Epub 2024 Aug 31. PMID: 39222737 Clinical Trial.
The Seventh World Symposium on Pulmonary Hypertension: our journey to Barcelona.
Authors: Humbert M, Galiè N, Rubin LJ, Simonneau G, McLaughlin VV. Eur Respir J. 2024 Oct 31;64(4):2401222. doi: 10.1183/13993003.01222-2024. Print 2024 Oct. PMID: 39209470 Free PMC article.
Clinical trial design, end-points, and emerging therapies in pulmonary arterial hypertension.
Authors: Weatherald J, Fleming TR, Wilkins MR, Cascino TM, Psotka MA, Zamanian R, Seeger W, Galiè N, Gomberg-Maitland M. Eur Respir J. 2024 Oct 31;64(4):2401205. doi: 10.1183/13993003.01205-2024. Print 2024 Oct. PMID: 39209468 Free PMC article. Review.
Prognostic role of haemodynamics at follow-up in patients with pulmonary arterial hypertension: a challenge to current European Society of Cardiology/European Respiratory Society risk tools.
Authors: Dardi F, Guarino D, Ballerini A, Bertozzi R, Donato F, Cennerazzo F, Salvi M, Nardi E, Magnani I, Manes A, Galiè N, Palazzini M. ERJ Open Res. 2024 Aug 5;10(4):00225-2024. doi: 10.1183/23120541.00225-2024. eCollection 2024 Jul. PMID: 39104950 Free PMC article.
ANMCO/SIC Consensus statement on pulmonary arterial hypertension].
Authors: Vatrano M, Manzi G, Picariello C, D’Alto M, Enea I, Ghio S, Caravita S, Argiento P, Garascia A, Vitulo P, Gabrielli D, Agostoni P, Corda M, Sinagra G, Grimaldi M, Scelsi L, Badagliacca R, D’Agostino C, Perrone Filardi P, Colivicchi F, Indolfi C, Roncon L, Galiè N, Oliva F, Vizza CD. G Ital Cardiol (Rome). 2024 Mar;25(3):192-201. doi: 10.1714/4209.42007. PMID: 38410902 Italian.
Comparison of cardiac magnetic resonance imaging, functional and haemodynamic variables in pulmonary arterial hypertension: insights from REPAIR.
Authors: Kiely DG, Channick R, Flores D, Galiè N, MacDonald G, Marcus JT, Mitchell L, Peacock A, Rosenkranz S, Tawakol A, Torbicki A, Vonk Noordegraaf A, Swift AJ. ERJ Open Res. 2024 Feb 12;10(1):00547-2023. doi: 10.1183/23120541.00547-2023. eCollection 2024 Jan. PMID: 38348238 Free PMC article.
Chronic thromboembolic pulmonary disease.
Authors: Kim NH, D’Armini AM, Delcroix M, Jaïs X, Jevnikar M, Madani MM, Matsubara H, Palazzini M, Wiedenroth CB, Simonneau G, Jenkins DP. Eur Respir J. 2024 Oct 31;64(4):2401294. doi: 10.1183/13993003.01294-2024. Print 2024 Oct. PMID: 39209473
Embracing the challenges of neonatal and paediatric pulmonary hypertension.
Authors: Ivy D, Rosenzweig EB, Abman SH, Beghetti M, Bonnet D, Douwes JM, Manes A, Berger RMF. Eur Respir J. 2024 Oct 31;64(4):2401345. doi: 10.1183/13993003.01345-2024. Print 2024 Oct. PMID: 39209483
Risk stratification and treatment goals in pulmonary arterial hypertension.
Authors: Dardi F, Boucly A, Benza R, Frantz R, Mercurio V, Olschewski H, Rådegran G, Rubin LJ, Hoeper MM. Eur Respir J. 2024 Oct 31;64(4):2401323. doi: 10.1183/13993003.01323-2024. Print 2024 Oct. PMID: 39209472
Relevance of comorbidities on initial combination therapy in pulmonary arterial hypertension.
Authors: Lombardi C, Manzi G, Mercurio V, Mulè M, Paciocco G, Papa S, Romeo E, Scelsi L, Stolfo D, Vitulo P, Vizza CD. ERJ Open Res. 2022 Nov 7;8(4):00298-2022. doi: 10.1183/23120541.00298-2022. PMID: 36382240; PMCID: PMC9638831.
Working group on congenital heart disease, cardiovascular prevention in paediatric age of the Italian Society of Cardiology (SIC). Primary and secondary paediatric hypertension.
Authors: Bassareo PP, Calcaterra G, Sabatino J, Oreto L, Ciliberti P, Perrone M, Martino F, D’Alto M, Chessa M, DI Salvo G, Guccione P; J Cardiovasc Med (Hagerstown). 2023 Apr 1;24 (Suppl 1):e77-e85. doi: 10.2459/JCM.0000000000001432. PMID: 37052224.
Tricuspid Regurgitation: Right Ventricular Volume Versus Pressure Load.
Authors: Naeije R, Tello K, D’Alto M. Curr Heart Fail Rep. 2023 Apr 26. doi: 10.1007/s11897-023-00599-w. Epub ahead of print. PMID: 37099262.
Raising the bar: triple therapy for pulmonary arterial hypertension associated with congenital heart disease.
Authors: D’Alto M, Bassareo PP. Heart. 2023 Oct 30:heartjnl-2023-323462. doi: 10.1136/heartjnl-2023-323462. Epub ahead of print. PMID: 37903554.
RIGHT Heart International NETwork (RIGHT-NET) Investigators. Exercise Stress Echocardiography of the Right Ventricle and Pulmonary Circulation.
Authors: Gargani L, Pugliese NR, De Biase N, Mazzola M, Agoston G, Arcopinto M, Argiento P, Armstrong WF, Bandera F, Cademartiri F, Carbone A, Castaldo R, Citro R, Cocchia R, Codullo V, D’Alto M, D’Andrea A, Douschan P, Fabiani I, Ferrara F, Franzese M, Frumento P, Ghio S, Grünig E, Guazzi M, Kasprzak JD, Kolias T, Kovacs G, La Gerche A, Limogelli G, Marra AM, Matucci-Cerinic M, Mauro C, Moreo A, Pratali L, Ranieri B, Rega S, Rudski L, Saggar R, Salzano A, Serra W, Stanziola AA, Vannan MA, Voilliot D, Vriz O, Wierzbowska-Drabik K, Cittadini A, Naeije R, Bossone E; J Am Coll Cardiol. 2023 Nov 21;82(21):1973-1985. doi: 10.1016/j.jacc.2023.09.807. PMID: 37968015.
Contribution of pressure and flow changes to resistance reduction after pulmonary arterial hypertension treatment: a meta-analysis of 3898 patients.
Authors: Farmakis IT, Baroutidou A, Patsiou V, Arvanitaki A, Doundoulakis I, Hobohm L, Zafeiropoulos S, Konstantinides SV, D’Alto M, Badagliacca R, Giannakoulas G. ERJ Open Res. 2024 Jan 22;10(1):00706-2023. doi: 10.1183/23120541.00706-2023. PMID: 38259812; PMCID: PMC10801731.
The Unveiled Triad: Clinical, Radiological and Pathological Insights into Hypersensitivity Pneumonitis.
Authors: Rea G, Bocchino M, Lieto R, Ledda RE, D’Alto M, Sperandeo M, Lucci R, Pasquinelli P, Sanduzzi Zamparelli S, Bocchini G, Valente T, Sica G. J Clin Med. 2024 Jan 30;13(3):797. doi: 10.3390/jcm13030797. PMID: 38337490; PMCID: PMC10856167.
Right heart failure as a cause of pulmonary congestion in pulmonary arterial hypertension.
Authors: D’Alto M, Di Maio M, Argiento P, Romeo E, Rea G, Liccardo B, Del Giudice C, Vergara A, Caiazza E, Del Vecchio GE, Di Vilio A, Gargani L, D’Andrea A, Bossone E, Golino P, Picano E, Naeije R. Eur J Heart Fail. 2024 Feb 26. doi: 10.1002/ejhf.3172. Epub ahead of print. PMID: 38404257.
Documento di consenso ANMCO/SIC sull’ipertensione arteriosa polmonare [ANMCO/SIC Consensus statement on pulmonary arterial hypertension].
Authors: Vatrano M, Manzi G, Picariello C, D’Alto M, Enea I, Ghio S, Caravita S, Argiento P, Garascia A, Vitulo P, Gabrielli D, Agostoni P, Corda M, Sinagra G, Grimaldi M, Scelsi L, Badagliacca R, D’Agostino C, Perrone Filardi P, Colivicchi F, Indolfi C, Roncon L, Galiè N, Oliva F, Vizza CD. G Ital Cardiol (Rome). 2024 Mar;25(3):192-201. Italian. doi: 10.1714/4209.42007. PMID: 38410902.
Gaps in evidence in the management of patients with intermediate-risk pulmonary arterial hypertension: Considerations following the ESC/ERS 2022 guidelines.
Authors: D’Alto M, Badagliacca R, Airò E, Ameri P, Argiento P, Garascia A, Lombardi CM, Mulè M, Raineri C, Scelsi L, Vizza CD, Ghio S. Vascul Pharmacol. 2024 Apr 18:107374. doi: 10.1016/j.vph.2024.107374. Epub ahead of print. PMID: 38642596.
Endothelial Function in Pulmonary Arterial Hypertension: From Bench to Bedside.
Authors: Correale M, Chirivì F, Bevere EML, Tricarico L, D’Alto M, Badagliacca R, Brunetti ND, Vizza CD, Ghio SJ Clin Med. 2024 Apr 22;13(8):2444. doi: 10.3390/jcm13082444. PMID: 38673717.
Relevance of Echocardiography-Derived Phenotyping in Pulmonary Arterial Hypertension Patients Treated with Initial Oral Combination Therapy.
Authors: Badagliacca R, Ghio S, D’Alto M, Ameri P, Correale M, Filomena D, Raineri C, Stolfo D, Naeije R, Vizza CD; iPHNET group. Am J Respir Crit Care Med. 2024 May 31. doi: 10.1164/rccm.202402-0431LE. Epub ahead of print. PMID: 38820124.
The pathophysiological approach: the best for phenotyping patients with pulmonary arterial hypertension.
Authors: Manzi G, Badagliacca R, Filomena D, D’Alto M, Vizza CD. Clin Res Cardiol. 2024 Jun 25. doi: 10.1007/s00392-024-02479-2. Epub ahead of print. PMID: 38918203.
Right ventricular phenotyping in incident patients with idiopathic pulmonary arterial hypertension.
Authors: Ghio S, Badagliacca R, D’Alto M, Scelsi L, Argiento P, Brunetti ND, Casu G, Cedrone N, Confalonieri M, Corda M, Correale M, D’Agostino C, De Tommasi E, Filomena D, Galgano G, Greco A, Grimaldi M, Lombardi C, Madonna R, Manzi G, Mercurio V, Mihai A, Mulè M, Paciocco G, Papa S, Recchioni T, Romaniello A, Romeo E, Stolfo D, Vitulo P, Benza RL, Vizza CD; italian Pulmonary Hpertension NETwork (iPHNET). J Heart Lung Transplant. 2024 Jun 26:S1053-2498(24)01698-X. doi: 10.1016/j.healun.2024.06.003. Epub ahead of print. PMID: 38942159.
Pathophysiology of the right ventricle and its pulmonary vascular interaction.
Authors: Hemnes AR, Celermajer DS, D’Alto M, Haddad F, Hassoun PM, Prins KW, Naeije R, Vonk Noordegraaf A. Eur Respir J. 2024 Aug 29:2401321. doi: 10.1183/13993003.01321-2024. Epub ahead of print. PMID: 39209482.
Improved Risk Prediction Using a Refined European Guidelines Instrument in Pulmonary Arterial Hypertension Related to Congenital Heart Disease.
Authors: van Dissel AC, D’Alto M, Farro A, Mathijssen H, Post MC, Bassareo PP, van Dijk APJ, Mulder BJM, Bouma BJAm J Cardiol. 2024 Sep 2:S0002-9149(24)00627-1. doi: 10.1016/j.amjcard.2024.08.027. Epub ahead of print. PMID: 39233061.
Gaps in evidence in the treatment of prevalent patients with pulmonary arterial hypertension at intermediate risk: An expert consensus.
Authors: Manzi G, Benza RL, Argiento P, Casu G, Corda M, Correale M, D’Alto M, Galgano G, Garascia A, Ghio S, Gomberg-Maitland M, Mulé M, Paciocco G, Papa S, Prati D, Preston IR, Raineri C, Romeo E, Scelsi L, Stolfo D, Vitulo P, White RJ, Badagliacca R, Vizza CD. Vascul Pharmacol. 2024 Sep 10:107432. doi: 10.1016/j.vph.2024.107432. Epub ahead of print. PMID: 39265796.
Progression, Management, and Outcome of Aortic Valve Stenosis in Systemic Sclerosis: A Case Series.
Authors: Vergara A, Orlando A, Caiazza E, Vettori S, Cuomo G, Argiento P, Romeo E, Franzese R, Sarubbi B, D’Alto M. J Cardiovasc Dev Dis 2024;11(9):274. doi: 10.3390/jcdd11090274. PMID: 39330332.
A pulmonary hypertension targeted algorithm to improve referral to right heart catheterization: A machine learning approach.
Authors: Argiento P, D’Agostino A, Castaldo R, Franzese M, Mazzola M, Grünig E, Saldamarco L, Valente V, Schiavo A, Maffei E, Lepre D, Cittadini A, Bossone E, D’Alto M, Gargani L, Marra AM. Comput Struct Biotechnol J. 2024 Nov 22;24:746-753. doi: 10.1016/j.csbj.2024.11.031. PMID: 39687751; PMCID: PMC11648641.
Pulmonary arterial hypertension with left to right shunts: When to treat and/or close?
Authors: D’Alto M, Romeo E, Argiento P, Vergara A, Caiazza E, Orlando A, Franzese R, Scognamiglio G, Sarubbi B, Dimopoulos K. Int J Cardiol Congenit Heart Dis. 2024 Jul 11;17:100526. doi: 10.1016/j.ijcchd.2024.100526. PMID: 39711769; PMCID: PMC11657717.
High-resolution DNA methylation changes reveal biomarkers of heart failure with preserved ejection fraction versus reduced ejection fraction.
Authors: Benincasa G, Pepin ME, Russo V, Cacciatore F, D’Alto M, Argiento P, Romeo E, Chiappetti R, Laezza N, Wende AR, Schiattarella GG, Coscioni E, La Montagna A, Amarelli C, Maiello C, Golino P, Condorelli G, Napoli C. Basic Res Cardiol. 2024 Dec 27. doi: 10.1007/s00395-024-01093-7. Epub ahead of print. PMID: 39725721.
Echocardiography of the right heart in pulmonary arterial hypertension: insights from the ULTRA RIGHT VALUE study.
Authors: Lo Giudice F, Escribano-Subias P, Tello K, Kopec G, Ghio S, Giannakoulas G, D’Alto M, Filomena D, Manzi G, Orlando A, Greco A, Recchioni T, Yildiz S, López-Guarch CJ, Cruz-Utrilla A, Psochias P, Patsiou V, Stępniewski J, Jonas K, Scelsi L, Kremer N, Vergara A, Vizza CD, Naeije R, Badagliacca R. Eur Heart J Imaging Methods Pract. 2025 Jan 15;3(1):qyae121. doi: 10.1093/ehjimp/qyae121. PMID: 39816928; PMCID: PMC11733976.
Myo-inositol trispyrophosphate prevents right ventricular failure and improves survival in monocrotaline-induced pulmonary hypertension in the rat.
Authors: Torbicki, A., Kurzyna, M., Kieda, C., Piekiełko-Witkowska, A., & Mączewski, M. (2024). British journal of pharmacology, 181(20), 4050–4066. https://doi.org/10.1111/bph.16482
Discrepancy in the Diagnosis of Heart Failure With Preserved Ejection Fraction Between Supine Versus Upright Exercise Hemodynamic Testing.
Authors: Fudim M, Kittipibul V, Swavely A, Gray A, Mikitka J, Young E, Dobbin O, Radzom M, Fee J, Molinger J, Patterson B, Battista Perego G, Badano LP, Parati G, Vachiéry JL, Senni M, Lanzarone E, Previdi F, Paleari S, Baratto C, Caravita S. Circ Heart Fail. 2024 Dec;17(12):e012020. doi: 10.1161/CIRCHEARTFAILURE.124.012020. Epub 2024 Nov 8. PMID: 39513293.
Pulmonary arterial hypertension: Navigating the pathways of progress in diagnosis, treatment, and patient care
Authors: Azaredo Raposo M, Inácio Cazeiro, Plácido R, et al. Rev Port Cardiol. 2024 Jul 5:S0870-2551(24)00218-X. English, Portuguese. doi: 10.1016/j.repc.2024.03.004. Epub ahead of print. PMID: 38972452.
Chronic thromboembolic pulmonary hypertension: A comprehensive review of pathogenesis, diagnosis, and treatment strategies
Authors: Inácio Cazeiro D, Azaredo Raposo M, Plácido R, et al. Rev Port Cardiol. 2024 Jun 28:S0870-2551(24)00187-2. English, Portuguese.
Pulmonary Arterial Hypertension with Features of Venous Involvement: A Detective’s Task.
Authors: Cazeiro DI, Plácido R, Raposo MA, et al. Arq Bras Cardiol. 2024 Apr 29;121(4):e20230565. Portuguese, English.
Chronic fibrothrombotic deposits: a cause or consequence of pulmonary hypertension?
Authors: Brito J, Pinto FJ, Plácido R. Rev Esp Cardiol (Engl Ed). 2024 Jan 6:S18855857(24)00015-X. English, Spanish. doi: 10.1016/j.rec.2023.12.005.
A Challenging Case of Pulmonary Arterial Hypertension: Never Judge By Appearances
Authors: António P, António, M, Plácido R, et al. Int J Cardiovasc Sci. 2024; 37:e20230129. doi: 10.36660/ijcs.20230129
Emerging therapies and new directions in the treatment of pulmonary arterial hypertension.
Authors: Kopeć G, Skride A, Ereminiene E, Jansa P. Kardiol Pol 2025;83(1):18-26. doi: 10.33963/v.phj.104053. Epub 2025 Jan 2.
Pulmonary perfusion in long-term survivors of COVID-19-related severe acute respiratory distress syndrome treated by extracorporeal membrane oxygenation.
Authors: Miksová L, Dytrych V, Ptáčník V…Jansa P. Pull Circ 2024 Aug 25;14(3):e12431. doi: 10.1002/pul2.12431. eCollection 2024 Jul.
A roadmap for therapeutic discovery in pulmonary hypertension associated with left heart failure. A scientific statement of the Heart Failure Association (HFA) of the ESC and the ESC Working Group on Pulmonary Circulation & Right Ventricular Function
Authors: Ameri P, Mercurio V, Pollesello P, Anker MS, Backs J, Bayes-Genis A, Borlaug BA, Burkhoff D, Caravita S, Chan SY, de Man F, Giannakoulas G, González A, Guazzi M, Hassoun PM, Hemnes AR, Maack C, Madden B, Melenovsky V, Müller OJ, Papp Z, Pullamsetti SS, Rainer PP, Redfield MM, Rich S, Schiattarella GG, Skaara H, Stellos K, Tedford RJ, Thum T, Vachiery JL, van der Meer P, Van Linthout S, Pruszczyk P, Seferovic P, Coats AJS, Metra M, Rosano G, Rosenkranz S, Tocchetti CG.. Eur J Heart Fail. 2024;26:707-729. doi: 10.1002/ejhf.3236. Epub 2024 Apr 19. PMID: 38639017; PMCID: PMC11182487.
Early Alteration of Right Ventricle-Pulmonary Artery Coupling in Experimental Heart Failure With Preserved Ejection Fraction.
Authors: Hubesch G, Dewachter C, Chomette L, Hupkens E, Jespers P, Vegh G, Doppler M, Sheikh Mohammad U, Thiriard A, Remmelink M, Vachiéry JL, McEntee K, Dewachter L. J Am Heart Assoc. 2024;13:e032201. doi: 10.1161/JAHA.123.032201. Epub 2024 May 23. PMID: 38780193; PMCID: PMC11255620.
Pulmonary hypertension associated with left heart disease.
Authors: Maron BA, Bortman G, De Marco T, Huston JH, Lang IM, Rosenkranz SH, Vachiéry JL, Tedford RJ. Eur Respir J. 2024 Oct 31;64(4):2401344. doi: 10.1183/13993003.01344-2024. PMID: 39209478; PMCID: PMC11525340.
Highlights for the heart failure cardiologist from the 7th World Symposium on Pulmonary Hypertension: Are we out of the woods yet?
Authors: Cascino TM, Maron BA, Vachiery JL, McLaughlin VV, Tedford RJ. J Card Fail. 2024:S1071-9164(24)00414-7. doi: 10.1016/j.cardfail.2024.09.009. Epub ahead of print. PMID: 39341548.
A Belgian consensus on sotatercept for the treatment of pulmonary arterial hypertension. Authors: Vachiéry, J. L., Belge, C., Cools, B., Damen, A., Demeure, F., De Pauw, M., Delcroix, M. (2024). Acta Cardiologica, 1–6. https://doi.org/10.1080/00015385.2024.2408130
Right ventricular reserve in cardiopulmonary disease: A simultaneous hemodynamic and three-dimensional echocardiographic study.
Authors: Baratto C, Dewachter C, Forton K, Muraru D, Gagliardi MF, Tomaselli M, Gavazzoni M, Perego GB, Senni M, Bondue A, Badano LP, Parati G, Vachiéry JL, Caravita SJ Heart Lung Transplant. 2024 Dec 25:S1053-2498(24)02036-9. doi: 10.1016/j.healun.2024.12.022. Epub ahead of print. PMID: 39730080.
Discrepancy in the Diagnosis of Heart Failure With Preserved Ejection Fraction Between Supine Versus Upright Exercise Hemodynamic Testing
Authors: Fudim M, Kittipibul V, Swavely A, Gray A, Mikitka J, Young E, Dobbin O, Radzom M, Fee J, Molinger J, Patterson B, Battista Perego G, Badano LP, Parati G, Vachiéry JL, Senni M, Lanzarone E, Previdi F, Paleari S, Baratto C, Caravita S.. Circ Heart Fail. 2024 Dec;17(12):e012020. doi: 10.1161/CIRCHEARTFAILURE.124.012020. Epub 2024 Nov 8. PMID: 39513293. NA
Pulmonary hypertension during high-dose GM-CSF therapy of autoimmune pulmonary alveolar proteinosis.
Authors: Ataya A, Mitchel S, Carey B, Sippel J, McCarthy C, Trapnell BC. Pulm Circ. 2024; Nov 21;14(4):e70020. doi: 10.1002/pul2.70020. PMID: 39582774; PMCID: PMC11582013.
Riociguat in pulmonary arterial hypertension: Application of the 4-strata COMPERA 2.0 risk assessment tool in the PATENT studies.
Authors: Ghofrani HA. Respir Med. 2025 Jan;236:107910. Epub 2024 Dec 10.
Efficacy and safety of sotatercept across ranges of cardiac index in patients with pulmonary arterial hypertension: A pooled analysis of PULSAR and STELLAR.
Authors: Gomberg-Maitland M, Badesch DB, Gibbs JSR, Grünig E, Hoeper MM, Humbert M, Kopeć G, McLaughlin VV, Meyer G, Olsson KM, Preston IR, Rosenkranz S, Souza R, Waxman AB, Perchenet L, Strait J, Xing A, Johnson-Levonas AO, Cornell AG, de Oliveira Pena J, Ardeschir Ghofrani H. J Heart Lung Transplant. 2024 Dec 5: Epub ahead of print.
Association of Pulmonary Hypertension With Trastuzumab Emtansine: An Analysis of French Pulmonary Hypertension Registry and WHO Pharmacovigilance Database.
Authors: Poisson C, Hlavaty A, Favrolt N, Chaumais MC, Grynblat J, Jutant EM, Lechartier B, Maurac A, Mouillot P, Palat S, Rambach L, Antigny F, Cottin V, Beltramo G, Humbert M, Khouri C, Bonniaud P, Montani D. Chest. 2024 Nov 19: Epub ahead of print.
Transcriptome analyses reveal common immune system dysregulation in PAH patients and Kcnk3-deficient rats.
Authors: Ruffenach G, Le Ribeuz H, Dutheil M, El Jekmek K, Dumont F, Willer AS, Humbert M, Capuano V, Medzikovic L, Eghbali M, Montani D, Antigny F.. Pulm Circ. 2024 Oct 23;14(4):e12434.
MK-5475, an inhaled soluble guanylate cyclase stimulator, for treatment of pulmonary arterial hypertension: the INSIGNIA-PAH study. Authors: Humbert M, Hassoun PM, Chin KM, Bortman G, Patel MJ, La Rosa C, Fu W, Loureiro MJ, Hoeper MM. Eur Respir J. 2024 Nov 14;64(5):2401110.
Tailoring oral anticoagulant treatment in the era of multi-drug therapies for PAH and CTEPH. Tailoring oral anticoagulant treatment in the era of multi-drug therapies for PAH and CTEPH.
Authors: Escal J, Poenou G, Delavenne X, Bezzeghoud S, Mismetti V, Humbert M, Montani D, Bertoletti L. Blood Rev. 2024 Nov;68:101240.
Balloon pulmonary angioplasty for proximal chronic thromboembolic pulmonary hypertension in patients ineligible for pulmonary endarterectomy.
Authors: Issard J, Fadel E, Dolidon S, Gerardin B, Fabre D, Mitilian D, Mercier O, Jevnikar M, Jais X, Humbert M, Brenot P. Pulm Circ. 2024 Sep 3;14(3):e12432.
Treatment algorithm for pulmonary arterial hypertension.
Authors: Chin KM, Gaine SP, Gerges C, Jing ZC, Mathai SC, Tamura Y, McLaughlin VV, Sitbon O. Eur Respir J. 2024 Oct 31;64(4):2401325.
Definition, classification and diagnosis of pulmonary hypertension.
Authors: Kovacs G, Bartolome S, Denton CP, Gatzoulis MA, Gu S, Khanna D, Badesch D, Montani D. Eur Respir J. 2024 Oct 31;64(4):2401324.
Transplantation, bridging, and support technologies in pulmonary hypertension.
Authors: Savale L, Benazzo A, Corris P, Keshavjee S, Levine DJ, Mercier O, Davis RD, Granton JT. Eur Respir J. 2024 Oct 31;64(4):2401193.
Integrative Multiomics in the Lung Reveals a Protective Role of Asporin in Pulmonary Arterial Hypertension.
Authors: Hong J, Medzikovic L, Sun W, Wong B, Ruffenach G, Rhodes CJ, Brownstein A, Liang LL, Aryan L, Li M, Vadgama A, Kurt Z, Schwantes-An TH, Mickler EA, Gräf S, Eyries M, Lutz KA, Pauciulo MW, Trembath RC, Perros F, Montani D, Morrell NW, Soubrier F, Wilkins MR, Nichols WC, Aldred MA, Desai AA, Trégouët DA, Umar S, Saggar R, Channick R, Tuder RM, Geraci MW, Stearman RS, Yang X, Eghbali M.. Circulation. 2024 Oct 15;150(16):1268-1287.
Development and validation of a code-based algorithm using in-hospital medical records to identify patients with pulmonary arterial hypertension in a French healthcare database.
Authors: Jambon-Barbara C, Hlavaty A, Bernardeau C, Bouvaist H, Chaumais MC, Humbert M, Montani D, Cracowski JL, Khouri C. ERJ Open Res. 2024 Aug 12;10(4):00109-2024.
Unraveling the Impact of miR-146a in Pulmonary Arterial Hypertension Pathophysiology and Right Ventricular Function.
Authors: Santos-Gomes J, Mendes-Ferreira P, Adão R, Maia-Rocha C, Rego B, Poels M, Saint-Martin Willer A, Masson B, Provencher S, Bonnet S, Montani D, Perros F, Antigny F, Leite-Moreira AF, Brás-Silva C. Int J Mol Sci. 2024 Jul 24;25(15):8054.
Role of Exercise Hemodynamics in the Prediction of Pulmonary Arterial Hypertension in BMPR2 Mutation Carriers
Authors: Gerges C, Beurnier A, Jaïs X, Hervé P, Lau EMT, Girerd B, Günther S, Bouchachi A, Jevnikar M, Boucly A, Bogaard HJ, Simonneau G, Sitbon O, Savale L, Chemla D, Humbert M, Montani D. Chest. 2024 Nov;166(5):1173-1183.
Post-capillary pulmonary hypertension in heart failure: impact of current definition in the PH-HF multicentre study.
Authors: Fauvel C, Damy T, Berthelot E, Bauer F, Eicher JC, de Groote P, Trochu JN, Picard F, Renard S, Bouvaist H, Logeart D, Roubille F, Sitbon O, Lamblin N.. Eur Heart J. 2024 Sep 14;45(35):3274-3288.
Deep phenotyping of unaffected carriers of pathogenic BMPR2 variants screened for pulmonary arterial hypertension.
Authors: Tóth EN, Celant LR, Niglas M, Jansen S, Tramper J, Baxan N, Ashek A, Wessels JN, Marcus JT, Meijboom LJ, Houweling AC, Nossent EJ, Aman J, Grynblat J, Perros F, Montani D, Vonk Noordegraaf A, Zhao L, de Man FS, Bogaard HJ. Eur Respir J. 2024 Oct 3;64(4):2400442.
Pulmonary Hypertension Induced by Right Pulmonary Artery Occlusion: Hemodynamic Consequences of Bmpr2 Mutation.
Authors: Todesco A, Grynblat J, Akoumia KKF, Bonnet D, Mendes-Ferreira P, Morisset S, Chemla D, Levy M, Méot M, Malekzadeh-Milani SG, Tielemans B, Decante B, Vastel-Amzallag C, Habert P, Ghigna MR, Humbert M, Montani D, Boulate D, Perros F.. J Am Heart Assoc. 2024 Jul 16;13(14):e034621.
Unmet need in pulmonary hypertension-associated interstitial lung disease (PH-ILD): a clinician survey of real-world management of PH-ILD in Europe.
Authors: Montani D, Cifrián JM, Rojo RP, Nunes H, Meloni F, Ghio S, Cannon J, Günther A, Gálvez García H, Delgado MF, Jeanneret GSB, Howard L. ERJ Open Res. 2024 Jul 8;10(4):00039-2024.
Evidence and unresolved questions in pulmonary hypertension: Insights from the 5th French Pulmonary Hypertension Network Meeting.
Authors: Boucly A, Bertoletti L, Fauvel C, Dewavrin MG, Gerges C, Grynblat J, Guignabert C, Hascoet S, Jaïs X, Jutant EM, Lamblin N, Meyrignac O, Riou M, Savale L, Tromeur C, Turquier S, Valentin S, Simonneau G, Humbert M, Sitbon O, Montani D; on behalf on the French PH Network. Respir Med Res. 2024 Nov;86:101123.
Medication adherence, related factors and outcomes among patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension: a systematic review.
Authors: Le Bozec A, Korb-Savoldelli V, Boiteau C, Dechartres A, Al Kahf S, Sitbon O, Montani D, Jaïs X, Guignabert C, Humbert M, Savale L, Chaumais MC. Eur Respir Rev. 2024 Jul 3;33(173):240006.
Effectiveness of Sotatercept and Approved Add-On Pulmonary Arterial Hypertension Therapies: A Systematic Review and Network Meta-Analysis.
Authors: Pitre T, Desai K, Mah J, Zeraatkar D, Humbert M. Comparative Ann Am Thorac Soc. 2024 Aug;21(8):1194-1203.
Characteristics and outcomes of gemcitabine-associated pulmonary hypertension.
Authors: Mouillot P, Favrolt N, Khouri C, Grandvuillemin A, Chaumais MC, Schenesse D, Seferian A, Jais X, Savale L, Beltramo G, Sitbon O, Cracowski JL, Humbert M, Georges M, Bonniaud P, Montani D. ERJ Open Res. 2024 May 20;10(3):00654-2023.
Percutaneous atrial septal defect closure in patients with pulmonary arterial hypertension.
Authors: Valdeolmillos E, Foray C, Albenque G, Batteux C, Petit J, Lecerf F, Jaïs X, Sitbon O, Montani D, Savale L, Humbert M, Hascoët S. Eur Respir J. 2024 May 30;63(5):2301649.
Orai1/STIMs modulators in pulmonary vascular diseases.
Authors: Saint-Martin Willer A, Montani D, Capuano V, Antigny F. Cell Calcium. 2024 Jul;121:102892.
Is Higher Dose of Inhaled Treprostinil Truly Superior to Lower Dose of Inhaled Treprostinil in Patients With Pulmonary Hypertension Associated With Interstitial Lung Diseases?
Authors: Genecand L, Lador F, Montani D, Perneger T. Chest. 2024 May;165(5):e161.
Seralutinib in adults with pulmonary arterial hypertension (TORREY): a randomised, double-blind, placebo-controlled phase 2 trial.
Authors: Frantz RP, McLaughlin VV, Sahay S, Escribano Subías P, Zolty RL, Benza RL, Channick RN, Chin KM, Hemnes AR, Howard LS, Sitbon O, Vachiéry JL, Zamanian RT, Cravets M, Roscigno RF, Mottola D, Osterhout R, Bruey JM, Elman E, Tompkins CA, Parsley E, Aranda R, Zisman LS, Ghofrani HA; TORREY Study Investigators. Lancet Respir Med. 2024 Jul;12(7):523-534.
Characteristics and outcomes of patients developing pulmonary hypertension associated with proteasome inhibitors.
Authors: Grynblat J, Khouri C, Hlavaty A, Jaïs X, Savale L, Chaumais MC, Kularatne M, Jevnikar M, Boucly A, Antigny F, Perros F, Simonneau G, Sitbon O, Humbert M, Montani D; and the French PH Network PULMOTENSION Investigators. Eur Respir J. 2024 Jun 28;63(6):2302158.
Risk stratification refinements with inclusion of haemodynamic variables at follow-up in patients with pulmonary arterial hypertension.
Authors: Boucly A, Beurnier A, Turquier S, Jevnikar M, de Groote P, Chaouat A, Cheron C, Jaïs X, Picard F, Prévot G, Roche A, Solinas S, Cottin V, Bauer F, Montani D, Humbert M, Savale L, Sitbon O; PulmoTension Network. Eur Respir J. 2024 Sep 5;64(3):2400197.
Role of KCNK3 Dysfunction in Dasatinib-associated Pulmonary Arterial Hypertension and Endothelial Cell Dysfunction.
Authors: Ribeuz HL, Willer AS, Chevalier B, Sancho M, Masson B, Eyries M, Jung V, Guerrera IC, Dutheil M, Jekmek KE, Laubry L, Carpentier G, Perez-Vizcaino F, Tu L, Guignabert C, Chaumais MC, Péchoux C, Humbert M, Hinzpeter A, Mercier O, Capuano V, Montani D, Antigny F. Am J Respir Cell Mol Biol. 2024 Jul;71(1):95-109
IMPAHCT: A randomized phase 2b/3 study of inhaled imatinib for pulmonary arterial hypertension.
Authors: Gillies H, Chakinala MM, Dake BT, Feldman JP, Hoeper MM, Humbert M, Jing ZC, Langley J, McLaughlin VV, Niven RW, Rosenkranz S, Zhang X, Hill NS. Pulm Circ. 2024 Mar 25;14(1):e12352.
ERS International Congress 2023: highlights from the Pulmonary Vascular Diseases Assembly.
Authors: Cullivan S, Boucly A, Jevnikar M, Lechartier B, Ulrich S, Bertoletti L, Sitbon O, Vonk-Noordegraaf A, Bokan A, Park DH, Genecand L, Guiot J, Jutant EM, Piccari L, Lichtblau M. ERJ Open Res. 2024 Feb 26;10(1):00847-2023.
4D flow cardiac MRI to assess pulmonary blood flow in patients with pulmonary arterial hypertension associated with congenital heart disease.
Authors: Valdeolmillos E, Sakhi H, Tortigue M, Audié M, Isorni MA, Lecerf F, Sitbon O, Montani D, Jais X, Savale L, Humbert M, Azarine A, Hascoët S. Diagn Interv Imaging. 2024 Jul-Aug;105(7-8):266-272.
Pulmonary Veno-Occlusive Disease and Pulmonary Fibrosis in a Family with EIF2AK4 Pathogenic Variants.
Authors: Vetel K, Eyries M, Savale L, Kanengiesser C, Borie R, Humbert M, Montani D. Am J Respir Cell Mol Biol. 2024 Feb;70(2):143-145.
Outcomes of listing for lung and heart-lung transplantation in pulmonary hypertension: comparative experience in France and the UK.
Authors: Pradère P, Le Pavec J, Bos S, Pozza A, Nair A, Meachery G, Lordan J, Humbert M, Mercier O, Fadel E, Savale L, Fisher AJ. ERJ Open Res. 2024 Jan 22;10(1):00521-2023.
Sarcoidosis-Associated Pulmonary Hypertension.
Authors: Savale L, Dorfmüller P, Boucly A, Jaïs X, Lacoste-Palasset T, Jevnikar M, Seferian A, Humbert M, Sitbon O, Montani D. Clin Chest Med. 2024 Mar;45(1):185-197.
Pulmonary veno-occlusive disease: illustrative cases and literature review.
Authors: Lechartier B, Boucly A, Solinas S, Gopalan D, Dorfmüller P, Radonic T, Sitbon O, Montani D. Eur Respir Rev. 2024 Jan 17;33(171):230156.
Outcomes and risk assessment in pulmonary veno-occlusive disease.
Authors: Boucly A, Solinas S, Beurnier A, Jaïs X, Keddache S, Eyries M, Seferian A, Jevnikar M, Roche A, Bulifon S, Bourdin A, Chaouat A, Cottin V, Bertoletti L, Savale L, Humbert M, Sitbon O, Montani D. ERJ Open Res. 2024 Jan 15;10(1):00612-2023.
Allele-specific control of rodent and human lncRNA KMT2E-AS1 promotes hypoxic endothelial pathology in pulmonary hypertension.
Authors: Tai YY, Yu Q, Tang Y, Sun W, Kelly NJ, Okawa S, Zhao J, Schwantes-An TH, Lacoux C, Torrino S, Al Aaraj Y, El Khoury W, Negi V, Liu M, Corey CG, Belmonte F, Vargas SO, Schwartz B, Bhat B, Chau BN, Karnes JH, Satoh T, Barndt RJ, Wu H, Parikh VN, Wang J, Zhang Y, McNamara D, Li G, Speyer G, Wang B, Shiva S, Kaufman B, Kim S, Gomez D, Mari B, Cho MH, Boueiz A, Pauciulo MW, Southgate L, Trembath RC, Sitbon O, Humbert M, Graf S, Morrell NW, Rhodes CJ, Wilkins MR, Nouraie M, Nichols WC, Desai AA, Bertero T, Chan SY. Sci Transl Med. 2024 Jan 10;16(729):eadd2029.
Thirty years of surgical management of pediatric pulmonary hypertension: Mid-term outcomes following reverse Potts shunt and transplantation.
Authors: Valdeolmillos E, Le Pavec J, Audié M, Savale L, Jais X, Montani D, Sitbon O, Feuillet S, Mercier O, Petit J, Humbert M, Fadel E, Belli E, Hascoët S. J Thorac Cardiovasc Surg. 2024 Sep;168(3):943-954. doi: 10.1016/j.jtcvs.2023.11.045.
Central role of ubiquitin-specific protease 8 in leptin signaling pathway in pulmonary arterial hypertension.
Authors: Jutant EM, Chelgham MK, Ottaviani M, Thuillet R, Le Vely B, Humbert M, Guignabert C, Tu L, Huertas A. J Heart Lung Transplant. 2024 Jan;43(1):120-133. doi: 10.1016/j.healun.2023.09.003.
The Pulmonary Artery Pulsatility Index Provides No Additional Prognostic Information in Pediatric Pulmonary Arterial Hypertension.
Authors: Smits FE, Lokhorst C, Haarman MG, Ploegstra MJ, Berger RMF, Douwes JM. Children (Basel). 2024 Sep 24;11(10):1152. doi: 10.3390/children11101152. PMID: 39457117; PMCID: PMC11506445.
Long-term outcome of children with newly diagnosed pulmonary arterial hypertension: results from the global TOPP registry.
Authors: Ploegstra MJ, Ivy DD, Beghetti M, Bonnet D, Alehan D, Ablonczy L, et al. Eur Heart J Qual Care Clin Outcomes 2024;10(1):66-76. DOI: 10.1093/ehjqcco/qcad020.
Deterioration in Renal Function in Patients With a Fontan Circulation and Association With Mortality.
Authors: van Hassel G, Groothof D, Douwes JM, Hoendermis ES, Liem ET, Willems TP, Ebels T, Voors AA, Bakker SJL, Berger RMF, van Melle JP. JACC Adv. 2024 Nov 19;3(12):101399. doi: 10.1016/j.jacadv.2024.101399. PMID: 39629062; PMCID: PMC11612357.
Embracing the challenges of neonatal and paediatric pulmonary hypertension.
Authors: Ivy D, Rosenzweig EB, Abman SH, Beghetti M, Bonnet D, Douwes JM, Manes A, Berger RMF. Eur Respir J. 2024 Oct 31;64(4):2401345. doi: 10.1183/13993003.01345-2024. PMID: 39209483; PMCID: PMC11525338.
Diagnostic yield and therapeutic implications of 25 years of specialized pediatric Marfan clinic.
Authors: Accord RE, Koster C, Dulfer E, du Marchie Sarvaas GJ, Maass SWMC, Berger RMF, van den Berg MP.. Eur J Pediatr. 2024 Nov 18;184(1):25. doi: 10.1007/s00431-024-05857-0. PMID: 39557716.
Population Pharmacokinetics and Target Attainment of Allopurinol and Oxypurinol Before, During, and After Cardiac Surgery with Cardiopulmonary Bypass in Neonates with Critical Congenital Heart Disease.
Authors: Chu WY, Nijman M, Stegeman R, Breur JMPJ, Jansen NJG, Nijman J, van Loon K, Koomen E, Allegaert K, Benders MJNL, Dorlo TPC, Huitema ADR; CRUCIAL trial consortium. Clin Pharmacokinet. 2024 Aug;63(8):1205-1220. doi: 10.1007/s40262-024-01401-3. Epub 2024 Aug 15. PMID: 39147988; PMCID: PMC11343829.
Iron deficiency in patients with a Fontan circulation and its impact on exercise capacity.
Authors: van Hassel G, Rivrud SCS, Timmerman FJ, van der Meer P, Hoendermis ES, Liem ET, Berger RMF, van Melle JP. Open Heart. 2024 Jun 5;11(1):e002693. doi: 10.1136/openhrt-2024-002693. PMID: 38839367; PMCID: PMC11163596.
Application of a modified clinical classification for pulmonary arterial hypertension associated with congenital heart disease in children: emphasis on atrial septal defects and transposition of the great arteries. An analysis from the TOPP registry.
Authors: Wacker J, Humpl T, Berger RMF, Ivy D, Bowers D, Bonnet D, Beghetti M. Front Cardiovasc Med. 2024 Feb 2;11:1344014. doi: 10.3389/fcvm.2024.1344014. PMID: 38370158; PMCID: PMC10870771.
2023
Metabolomics in Pulmonary Hypertension-A Useful Tool to Provide Insights into the Dark Side of a Tricky Pathology.
Authors: Bassareo PP, D’Alto M.Int J Mol Sci. 2023 Aug 25;24(17):13227. doi: 10.3390/ijms241713227.PMID: 37686034 Free PMC article. Review.
Lung Ultrasound, Echocardiography, and Fluid Challenge for the Differential Diagnosis of Pulmonary Hypertension
Authors: D’Alto M, Liccardo B, Di Maio M, Del Giudice C, Romeo E, Argiento P, Renon F, Vergara A, Di Vilio A, Caiazza E, Bossone E, Rea G, D’Andrea A, Gargani L, Golino P, Naeije R.J Am Soc Echocardiogr. 2023 Aug 5:S0894-7317(23)00411-X. doi: 10.1016/j.echo.2023.07.010. Online ahead of print.PMID: 37544385
Beyond Visual Interpretation: Quantitative Analysis and Artificial Intelligence in Interstitial Lung Disease Diagnosis “Expanding Horizons in Radiology”
Authors: Rea G, Sverzellati N, Bocchino M, Lieto R, Milanese G, D’Alto M, Bocchini G, Maniscalco M, Valente T, Sica G.Diagnostics (Basel). 2023 Jul 10;13(14):2333. doi: 10.3390/diagnostics13142333.PMID: 37510077 Free PMC article. Review.
Prevalence and clinical significance of right ventricular pulmonary arterial uncoupling in cardiac amyloidosis.
Authors: Palmiero G, Monda E, Verrillo F, Dongiglio F, Caiazza M, Rubino M, Lioncino M, Diana G, Vetrano E, Fusco A, Cirillo A, Mauriello A, Ciccarelli G, Ascione L, De Rimini ML, D’Alto M, Cerciello G, D’Andrea A, Golino P, Calabrò P, Bossone E, Limongelli G.Int J Cardiol. 2023 Oct 1;388:131147. doi: 10.1016/j.ijcard.2023.131147. Epub 2023 Jul 7.PMID: 37423570
The heart of the matter: Right heart imaging indicators for treatment escalation in pulmonary arterial hypertension.
Authors: Forfia P, Benza R, D’Alto M, De Marco T, Elwing JM, Frantz R, Haddad F, Oudiz R, Preston IR, Rosenkranz S, Ryan J, Schilz R, Shlobin OA, Vachiery JL, Vizza CD, Noordegraaf AV, Sketch MR, Broderick M, McLaughlin V.Pulm Circ. 2023 May 21;13(2):e12240. doi: 10.1002/pul2.12240. eCollection 2023 Apr.PMID: 37222992 Free PMC article.
Multimodality imaging in decompensated heart failure.
Authors: D’Andrea A, Ilardi F, Palermi S, Riegler L, Miele T, Giallauria F, D’Alto M, Russo V, Cice G.Eur Heart J Suppl. 2023 Apr 26;25(Suppl C):C292-C300. doi: 10.1093/eurheartjsupp/suad052. eCollection 2023 May.PMID: 37125293 Free PMC article.
Tricuspid Regurgitation: Right Ventricular Volume Versus Pressure Load.
Authors: Naeije R, Tello K, D’Alto M.Curr Heart Fail Rep. 2023 Jun;20(3):208-217. doi: 10.1007/s11897-023-00599-w. Epub 2023 Apr 26.PMID: 37099262 Review.
Primary and secondary paediatric hypertension.
Authors: Bassareo PP, Calcaterra G, Sabatino J, Oreto L, Ciliberti P, Perrone M, Martino F, D’Alto M, Chessa M, DI Salvo G, Guccione P; Working group on congenital heart disease, cardiovascular prevention in paediatric age of the Italian Society of Cardiology (SIC).J Cardiovasc Med (Hagerstown). 2023 Apr 1;24(Suppl 1):e77-e85. doi: 10.2459/JCM.0000000000001432.PMID: 37052224 Review.
Idiopathic Pulmonary Arterial Hypertension in Paediatrics Represents Still a Serious Challenge: A Case Series Study.
Authors: Bassareo PP, Argiento P, McMahon CJ, Dunne E, Walsh KP, Russo MG, D’Alto M.Children (Basel). 2023 Mar 6;10(3):518. doi: 10.3390/children10030518.PMID: 36980076 Free PMC article.
European Society of Cardiology quality indicators for the care and outcomes of adults with pulmonary arterial hypertension. Developed in collaboration with the Heart Failure Association of the European Society of Cardiology.
Authors: Aktaa S, Gale CP, Brida M, Giannakoulas G, Kovacs G, Adir Y, Benza RL, Böhm M, Coats A, D’Alto M, Escribano-Subias P, Ferrari P, Galiè N, Gibbs JSR, Gin-Sing W, Hoeper MM, Humbert M, Lang IM, Maron BA, Meszaros G, Vonk Noordegraaf A, Price LC, Pepke-Zaba J, Rådegran G, Reis A, Sitbon O, Torbicki A, Ulrich S, Rosenkranz S, Delcroix M.Eur J Heart Fail. 2023 Apr;25(4):469-477. doi: 10.1002/ejhf.2830. Epub 2023 Apr 4.PMID: 36924171
Cardiovascular Complications of Down Syndrome: Scoping Review and Expert Consensus.
Authors: Dimopoulos K, Constantine A, Clift P, Condliffe R, Moledina S, Jansen K, Inuzuka R, Veldtman GR, Cua CL, Tay ELW, Opotowsky AR, Giannakoulas G, Alonso-Gonzalez R, Cordina R, Capone G, Namuyonga J, Scott CH, D’Alto M, Gamero FJ, Chicoine B, Gu H, Limsuwan A, Majekodunmi T, Budts W, Coghlan G, Broberg CS; for Down Syndrome International (DSi).Circulation. 2023 Jan 31;147(5):425-441. doi: 10.1161/CIRCULATIONAHA.122.059706. Epub 2023 Jan 30.PMID: 36716257 Free PMC article. Review
Imaging the right atrium in pulmonary hypertension: A systematic review and meta-analysis.
Authors: Richter MJ, Fortuni F, Alenezi F, D’Alto M, Badagliacca R, Brunner NW, van Dijk AP, Douschan P, Gall H, Ghio S, Giudice FL, Grünig E, Haddad F, Howard L, Rajagopal S, Stens N, Stolfo D, Thijssen DHJ, Vizza CD, Zamanian RT, Zhong L, Seeger W, Ghofrani HA, Tello K.J Heart Lung Transplant. 2023 Apr;42(4):433-446. doi: 10.1016/j.healun.2022.11.007. Epub 2022 Dec 9.PMID: 36610927 Review.
Impact of Hormonal-Anabolic Deficiencies in Idiopathic Pulmonary Arterial Hypertension.
Authors: Marra AM, D’Agostino A, Salzano A, Basili S, D’Alto M, Bossone E, Cittadini A, Vizza CD, Badagliacca R.Heart Fail Clin. 2023 Jan;19(1):115-123. doi: 10.1016/j.hfc.2022.09.001.PMID: 36435567 Review.
Right heart failure in left heart disease: imaging, functional, and biochemical aspects of right ventricular dysfunction.
Authors: Ghio S, Acquaro M, Agostoni P, Ambrosio G, Carluccio E, Castiglione V, Colombo D, D’Alto M, Delle Grottaglie S, Dini FL, Emdin M, Fortunato M, Guaricci AI, Jacoangeli F, Marra AM, Paolillo S, Papa S, Scajola LV, Correale M, Palazzuoli A.Heart Fail Rev. 2023 Jul;28(4):1009-1022. doi: 10.1007/s10741-022-10276-0. Epub 2022 Nov 16.PMID: 36385328 Review.
Mining the Plasma Proteome for Insights into the Molecular Pathology of Pulmonary Arterial Hypertension.
Authors: Harbaum L, Rhodes CJ, Wharton J, Lawrie A, Karnes JH, Desai AA, Nichols WC, Humbert M, Montani D, Girerd B, Sitbon O, Boehm M, Novoyatleva T, Schermuly RT, Ghofrani HA, Toshner M, Kiely DG, Howard LS, Swietlik EM, Gräf S, Pietzner M, Morrell NW, Wilkins MR; U.K. National Institute for Health Research BioResource Rare Diseases Consortium, U.K. Pulmonary Arterial Hypertension Cohort Study Consortium, and U.S. Pulmonary Arterial Hypertension Biobank Consortium. Am J Respir Crit Care Med. 2022 Jun 15;205(12):1449-1460. doi: 10.1164/rccm.202109-2106OC. PMID: 35394406; PMCID: PMC9875902.
Epigenetic Modification of the von Willebrand Factor Promoter Drives Platelet Aggregation on the Pulmonary Endothelium in Chronic Thromboembolic Pulmonary Hypertension.
Authors: Manz XD, Szulcek R, Pan X, Symersky P, Dickhoff C, Majolée J, Kremer V, Michielon E, Jordanova ES, Radonic T, Bijnsdorp IV, Piersma SR, Pham TV, Jimenez CR, Vonk Noordegraaf A, de Man FS, Boon RA, Voorberg J, Hordijk PL, Aman J, Bogaard HJ. Am J Respir Crit Care Med. 2022 Apr 1;205(7):806-818.doi: 10.1164/rccm.202109-2075OC. PMID: 35081007.
Too Hot to Handle?
Authors: Bogaard HJ, Aman J. Tyrosine Kinases and Endothelial Homeostasis in Pulmonary Arterial Hypertension: Am J Respir Cell Mol Biol. 2022 Aug;67(2):147-149. doi: 10.1165/rcmb.2022-0122ED. PMID: 35580152; PMCID: PMC9348559.
Endobronchial blocker use for massive, focal pulmonaryhemorrhage during pulmonary endarterectomy for CTEPH
Authors: Bonta PI, Tramper J, Eberl S, Tepaske R, Nossent EJ, Vonk-Noordgraaf A,Bogaard HJ, Symersky P.. J Heart Lung Transplant.2022 Aug;41(8):1130-1133. doi: 10.1016/j.healun.2022.04.006. Epub 2022 Apr 27.PMID: 35641423.
Nutritional status in pulmonary arterial hypertension.
Authors: Kwant CT, van der Horst FAL, Bogaard HJ, de Man FS, Vonk Noordegraaf A. Pulm Circ. 2022 Oct 1;12(4):e12173. doi: 10.1002/pul2.12173. PMID: 36568692; PMCID: PMC9768459.
Idiopathic pulmonary arterial hypertension patients with a high H<sub>2</sub>FPEF-score: Insights from the Amsterdam UMC PAH-cohort.
Authors: Kianzad A, van Wezenbeek J, Celant LR, Oosterveer FPT, Vonk Noordegraaf A, Meijboom LJ, de Man FS, Bogaard HJ, Handoko ML. . J Heart Lung Transplant. 2022 Aug;41(8):1075-1085.doi: 10.1016/j.healun.2022.05.007. Epub 2022 May 20. PMID: 3569760
A systematic review with meta-analysis of biomarkers for detection of pulmonary arterial hypertension.
Authors: Smits AJ, Botros L, Mol MAE, Ziesemer KA, Wilkins MR, Vonk Noordegraaf A, Bogaard HJ, Aman JERJ Open Res. 2022 May 30;8(2):00009-2022. doi: 10.1183/23120541.00009-2022. PMID: 35651362; PMCID: PMC9149393.
Distinct Platelet Ribonucleic Acid Signatures in Patients with Pulmonary Hypertension.
Authors: Smits AJ, Arkani M, In ‘t Veld SGJG, Huis In ‘t Veld AE, Sol N, Groeneveldt JA, Botros L, Braams NJ, Jansen SMA, Ramaker J, Zwaan K, Post E, Nossent EJ, Boonstra A, de Man FS, Vonk Noordegraaf A, Gomez-Arroyo J, Best MG, Wurdinger T, Bogaard HJ. Ann Am Thorac Soc. 2022 Oct;19(10):1650-1660. doi: 10.1513/AnnalsATS.202201-085OC. PMID: 35537078.
Clinical Characteristics of Patients Undergoing Right Heart Catheterizations in Community Hospitals.
Authors: Jansen SMA, Huis In ‘t Veld AE, Tolen PHCG, Jacobs W, Willemsen HM, Grotjohan HP, Waskowsky M, van der Maten J, van der Weerdt A, Hoekstra R, Pérez Matos AJ, Overbeek MJ, Mollema SA, El Bouazzaoui LHH, Vriend JWJ, Roorda JMM, de Nooijer R, van der Lee I, Voogel AJ, Post JC, Macken T, Aerts JM, van de Ven MJT, Bergman H, Bakker-de Boo M, de Boer RC, Vonk Noordegraaf A, de Man FS, Bogaard HJ. J Am Heart Assoc. 2022 Sep 6;11(17):e025143. doi: 10.1161/JAHA.121.025143. Epub 2022 Sep 5. PMID: 36062610;PMCID: PMC9496424.
BioResource for Translational Research–Rare Diseases; National Cohort Study of Idiopathic and Heritable PAH; PAH Biobank Enrolling Centers’ Investigators. First Genotype-Phenotype Study in TBX4 Syndrome: Gain-of-Function Mutations Causative for Lung Disease.
Authors: Prapa M, Lago-Docampo M, Swietlik EM, Montani D, Eyries M, Humbert M, Welch CL, Chung WK, Berger RMF, Bogaard HJ, Danhaive O, Escribano-Subías P, Gall H,Girerd B, Hernandez-Gonzalez I, Holden S, Hunt D, Jansen SMA, Kerstjens- Frederikse W, Kiely DG, Lapunzina P, McDermott J, Moledina S, Pepke-Zaba J,Polwarth GJ, Schotte G, Tenorio-Castaño J, Thompson AAR, Wharton J, Wort SJ, Megy K, Mapeta R, Treacy CM, Martin JM, Li W, Swift AJ, Upton PD, Morrell NW, Gräf S, Valverde D; NIHR Am J Respir Crit Care Med. 2022 Dec 15;206(12):1522-1533. doi: 10.1164/rccm.202203-0485OC. PMID: 35852389;PMCID: PMC9757087.
Noninvasive follow-up strategy after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension.
Authors: Ruigrok D, Handoko ML, Meijboom LJ, Nossent EJ, Boonstra A, Braams NJ, van Wezenbeek J, Tepaske R, Tuinman PR, Heunks LMA, Vonk Noordegraaf A, de Man FS, Symersky P, Bogaard HJ. ERJ Open Res. 2022 May 16;8(2):00564-2021. doi:10.1183/23120541.00564- 2021. PMID: 35586450;PMCID: PMC9108966.
Synthesis and preclinical evaluation of [<sup>11</sup>C]LR111 and [<sup>18</sup>F]EW-7197 as PET tracers of the activin-receptor like kinase-5.
Authors: Rotteveel L, Kurakula K, Kooijman EJM, Schuit RC, Verlaan M, Schreurs M, Beaino W, van Dinther MAH, Ten Dijke P, Lammertsma AA, Poot AJ, Bogaard HJ, Windhorst AD. Nucl Med Biol. 2022 Sep-Oct;112-113:9-19. doi: 10.1016/j.nucmedbio.2022.05.003.Epub 2022 May 24. PMID: 35660796.
Interplay of sex hormones and long-term right ventricular adaptation in a Dutch PAH-cohort.
Authors: van Wezenbeek J, Groeneveldt JA, Llucià-Valldeperas A, van der Bruggen CE, Jansen SMA, Smits AJ, Smal R, van Leeuwen JW, Remedios CD, Keogh A, Humbert M, Dorfmüller P, Mercier O, Guignabert C, Niessen HWM, Handoko ML, Marcus JT, Meijboom LJ, Oosterveer FPT, Westerhof BE, Heijboer AC, Bogaard HJ, Vonk Noordegraaf A, Goumans MJ, de Man FS. J Heart Lung Transplant. 2022 Apr;41(4):445-457. doi: 10.1016/j.healun.2021.11.004. Epub 2021 Nov 14. PMID: 35039146.
Metabolic profile in endothelial cells of chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension.
Authors: Smolders VFED, Rodríguez C, Blanco I, Szulcek R, Timens W, Piccari L, Roger Y, Hu X, Morén C, Bonjoch C, Sebastián L, Castellà M, Osorio J, Peinado VI, Bogaard HJ, Quax PHA, Cascante M, Barberà JA, Tura-Ceide O.. Sci Rep. 2022 Feb 10;12(1):2283. doi: 10.1038/s41598-022-06238-z. PMID: 35145193; PMCID: PMC8831561.
Author Correction: Biological heterogeneity in idiopathic pulmonary arterial hypertension identified through unsupervised transcriptomic profiling of whole blood.
Authors: Kariotis S, Jammeh E, Swietlik EM, Pickworth JA, Rhodes CJ, Otero P, Wharton J, Iremonger J, Dunning MJ, Pandya D, Mascarenhas TS, Errington N, Thompson AAR, Romanoski CE, Rischard F, Garcia JGN, Yuan JX, An TS, Desai AA, Coghlan G, Lordan J, Corris PA, Howard LS, Condliffe R, Kiely DG, Church C, Pepke-Zaba J, Toshner M, Wort S, Gräf S, Morrell NW, Wilkins MR, Lawrie A, Wang D; UK National PAH Cohort Study Consortium. Nat Commun. 2022 Nov 25;13(1):7276.doi: 10.1038/s41467-022-33381-y. Erratum for: Nat Commun. 2021 Dec 7;12(1):7104. PMID: 36433938; PMCID: PMC9700755.
Man FS. Right atrial function is associated with right venticular diastolic stiffness: RA-RV interaction in pulmonary arterial hypertension.
Authors: Wessels JN, Mouratoglou SA, van Wezenbeek J, Handoko ML, Marcus JT, Meijboom LJ, Westerhof BE, Bogaard HJ, Strijkers GJ, Vonk Noordegraaf A, de. Eur Respir J. 2022 Jun 23;59(6):2101454. doi: 10.1183/13993003.01454-2021. PMID: 34764180; PMCID:PMC9218241.
The Value of Passive Leg Raise During Right Heart Catheterization in Diagnosing Heart Failure With Preserved Ejection Fraction.
Authors: van de Bovenkamp AA, Wijkstra N, Oosterveer FPT, Vonk Noordegraaf A, Bogaard HJ, van Rossum AC, de Man FS, Borlaug BA, Handoko ML.. Circ Heart Fail. 2022 Apr;15(4):e008935. doi:10.1161/CIRCHEARTFAILURE.121.008935. Epub 2022 Mar 21. PMID: 35311526; PMCID:PMC9009844.
Inhibition of the prolyl isomerase Pin1 improves endothelial function and attenuates vascular remodelling in pulmonary hypertension by inhibiting TGF-β signalling. Angiogenesis.
Authors: Kurakula K, Hagdorn QAJ, van der Feen DE, Vonk Noordegraaf A, Ten Dijke P, de Boer RA, Bogaard HJ, Goumans MJ, Berger RMF. 2022Feb;25(1):99-112. doi: 10.1007/s10456-021-09812-7. Epub 2021 Aug 11. PMID:34379232; PMCID: PMC8813847.
Riociguat inhibits ultra-large VWF string formation on pulmonary artery endothelial cells from chronic thromboembolic pulmonary hypertension patients. Authors: Jujo Sanada T, Manz XD, Symersky P, Pan X, Yoshida K, Aman J, Bogaard HJ.. Pulm Circ. 2022 Oct 1;12(4):e12146. doi: 10.1002/pul2.12146. PMID: 36568694; PMCID: PMC9768460.
When right ventricular pressure meets volume: The impact of arrival time of reflected waves on right ventricle load in pulmonary arterial hypertension.
Authors: Fukumitsu M, Groeneveldt JA, Braams NJ, Bayoumy AA, Marcus JT, Meijboom LJ, de Man FS, Bogaard HJ, Vonk Noordegraaf A, Westerhof BE. . J Physiol. 2022 May;600(10):2327-2344. doi: 10.1113/JP282422. Epub 2022 Apr 27. PMID: 35421903;PMCID: PMC9321993.
Troponin dependent 30-day mortality in patients with acute pulmonary embolism
Authors: Sonne-Holm E, Winther-Jensen M, Bang LE, Køber L, Fosbøl E, Carlsen J, Kjaergaard J.. J Thromb Thrombolysis. 2023 Jul 24. doi: 10.1007/s11239-023-02864-0. Epub ahead of print. PMID: 37486553.
Calcium channel blockers and the risk of exacerbation in patients with Chronic Obstructive Pulmonary Disease: A nationwide study of 48,488 outpatients.
Authors: Ema Rastoder, Pradeesh Sivapalan, Josefin Eklöf, Imane Achir Alispahic, Alexander Jordan, Christian B. Laursen, Jørgen Vestbo, Christine Jenkins, Rune Nielsen, Per Bakke, Gustavo Fernandez-Romero, Daniel Modin, Niklas Dyrby Johansen, Filip Soeskov Davidovski, Tor Biering-Sørensen, Jørn Carlsen, Jens-Ulrik Jensen. Biomedicines 2023,11(7),1974; https://doi.org/10.3390/biomedicines110719747
Continuous long-term heart rate variability and risk assessment in pulmonary hypertension.
Authors: Andersen MØ, Diederichsen SZ, Svendsen JH, Carlsen J. Open Heart. 2023 Jun;10(1):e002302. doi: 10.1136/openhrt-2023-002302. PMID: 37385731; PMCID: PMC10314675.
Cardiac Characteristics of the First Two Waves of COVID-19 in Denmark and the Prognostic Value of Echocardiography: The ECHOVID-19 Study.
Authors: Christensen J, Davidoski FS, Skaarup KG, Lassen MCH, Alhakak AS, Sengeløv M, Nielsen AB, Johansen ND, Bundgaard H, Hassager C, Jabbari R, Carlsen J, Kirk O, Kristiansen OP, Nielsen OW, Ulrik CS, Sivapalan P, Gislason G, Iversen K, Jensen JUS, Schou M, Hviid A, Krause TG, Biering-Sørensen T. Cardiology. 2023;148(1):48-57. doi: 10.1159/000528308. Epub 2022 Dec 1. PMID: 36455539.
2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension.
Authors: Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, Carlsen J, Coats AJS, Escribano-Subias P, Ferrari P, Ferreira DS, Ghofrani HA, Giannakoulas G, Kiely DG, Mayer E, Meszaros G, Nagavci B, Olsson KM, Pepke-Zaba J, Quint JK, Rådegran G, Simonneau G, Sitbon O, Tonia T, Toshner M, Vachiery JL, Vonk Noordegraaf A, Delcroix M, Rosenkranz S; ESC/ERS Scientific Document Group. Eur Respir J. 2023 Jan 6;61(1):2200879. doi: 10.1183/13993003.00879-2022. Print 2023 Jan.
European Society of Cardiology quality indicators for the care and outcomes of adults with pulmonary arterial hypertension. Developed in collaboration with the Heart Failure Association of the European Society of Cardiology.
Authors: Suleman Aktaa, S., Gale, CP., Brida, M., Giannakoulas, G., Kovacs, G., Adir, Y., Benza, R.L., Böhm, M., Coats, A., D’Alto, M., Escribano-Subias, P., Ferrari, P., Galiè, N., Gibbs, J.S.R., Gin-Sing, W., Hoeper, M.M., Humbert, M., Lang, I.M., Maro, B.A.,Meszaros, G., V Noordegraa, A., Laura C Price, L.C., Pepke-Zaba, J., Göran Rådegran, G., Reis, A., Sitbon, O., Torbick, A.,Ulrich, S., Rosenkranz, S., Delcroix, M. Eur J Heart Fail 2023 Apr;25(4):469-477.
Quality of life and functional status after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: A Swedish single-center study.
Authors: Kallonen J, Corbascio M, Rådegran G, Bredin F, Sartipy U. Pulm Circ. Apr 1;13(2), 2023.
Adrenomedullin peptides and precursor levels in relation to haemodynamics and prognosis after heart transplantation.
Authors: Ahmed A, Kania K, Abdul Rahim H, Ahmed S, Rådegran G. . ESC Heart Fail. May 28, 2023.
Evaluation of the ESC/ERS derived three- and four-strata risk stratification models in PAH: introducing an internet-based risk stratification calculator.
Authors: Ahmed A., Ahmed S., Kempe, D. Rådegran, G., EHJ Open, 3, 1-14, 2023.
Plasma TRAIL and ANXA1 in diagnosis and prognostication of pulmonary arterial hypertension
Authors: M., Ahmed, A., Säleby, J., Ahmed, S., Hesselstrand, R., Rådegran, G. , Pulm. Circ. Epub July 25, 2023.
European Society of Cardiology quality indicators for the care and outcomes of adults with pulmonary arterial hypertension. Developed in collaboration with the Heart Failure Association of the European Society of Cardiology.
Authors:, Gale, CP, Brida, M., Giannakoulas, G., Kovacs, G., Adir, Y., Benza, R.L., Böhm, M, Andrew Coats, A., D’Alto, M., Escribano-Subias, P., Ferrari, P., Galiè, N., Gibbs, J.S.R., Gin-Sing, W., Hoeper, M.M., Marc Humbert, M., Lang, I.M., Maron, B.A., Meszaros, G., Vonk Noordegraaf, A., Price, L.C., Pepke-Zaba, J., Rådegran, G., Reis, A., Sitbon, O., Torbicki, A., Ulrich, S., Rosenkranz, S., Delcroix, M. . Eur J Heart Fail, Apr;25(4):469-477, 2023.
Structured evaluation of unclear dyspnea – of great importance to early identify patients with PAH and CTEPH and improve prognosis.
Authors: Ahmed S, Ahmed A, Rådegran G., Lakartidningen, 2022;119.
Risk assessment in PAH: A step towards clinical implementation based on the 2022 ESC/ERS PH guidelines.
Authors: Ahmed A., Ahmed S., Rådegran G. Circ. 2023.
Management of Pulmonary Hypertension Associated with Chronic Lung Disease.
Authors: Blanco I, Hernández-González F, García A, Torres-Castro R, Barberà JA. Semin Respir Crit Care Med. 2023 Jul 24. doi: 10.1055/s-0043-1770121. Online ahead of print. PMID: 37487524
Effects of Exercise Training on Circulating Biomarkers of Endothelial Function in Pulmonary Arterial Hypertension.
Authors: Rodríguez-Chiaradía DA, Khilzi K, Blanco I, Rodó-Pin A, Martin-Ontiyuelo C, Herranz Blasco A, Garcia-Lucio J, Molina L, Marco E, Barreiro E, Piccari L, Peinado VI, Garcia AR, Tura-Ceide O, Barberà JA. 2023 Jun 25;11(7):1822. doi: 10.3390/biomedicines11071822. PMID: 37509463
Mechanisms of hypoxaemia in severe pulmonary hypertension associated with COPD.
Authors: Piccari L, Blanco I, Torralba Y, Arismendi E, Gistau C, Ramírez A, Gimeno-Santos E, Roca J, Burgos F, Rodríguez-Roisín R, Barberà JA. Eur Respir J. 2023 Jul 20;62(1):2300463. doi: 10.1183/13993003.00463-2023. PMID: 37414421
SPHERE-HF Investigators. β3 adrenergic agonist treatment in chronic pulmonary hypertension associated with heart failure (SPHERE-HF): a double blind, placebo-controlled, randomized clinical trial.
Authors: García-Álvarez A, Blanco I, García-Lunar I, Jordà P, Rodriguez-Arias JJ, Fernández-Friera L, Zegri I, Nuche J, Gomez-Bueno M, Prat S, Pujadas S, Sole-Gonzalez E, Garcia-Cossio MD, Rivas M, Torrecilla E, Pereda D, Sanchez J, García-Pavía P, Segovia-Cubero J, Delgado JF, Mirabet S, Fuster V, Barberá JA, Ibañez B; Eur J Heart Fail. 2023 Mar;25(3):373-385. doi: 10.1002/ejhf.2745. Epub 2022 Dec 4. PMID: 36404400
Lung function in young adults born small for gestational age at term.
Authors: Vellvé K, Sepúlveda-Martínez Á, Rodríguez-López M, Crovetto F, Bernardino G, Burgos F, Faner R, Agustí À, Bijnens B, Gratacós E, Crispi F, Blanco I. 2023 Feb;28(2):183-186. doi: 10.1111/resp.14361. Epub 2022 Sep 29. PMID: 36172944
Survivin inhibition with YM155 ameliorates experimental pulmonary arterial hypertension.
Authors: Blanco I, Marquina M, Tura-Ceide O, Ferrer E, Ramírez AM, Lopez-Meseguer M, Callejo M, Perez-Vizcaino F, Peinado VI, Barberà JA. Front Pharmacol. 2023 Apr 24;14:1145994. doi: 10.3389/fphar.2023.1145994. eCollection 2023. PMID: 37188265
Atrophy signaling pathways in respiratory and limb muscles of guinea pigs exposed to chronic cigarette smoke: role of soluble guanylate cyclase stimulation. Authors: Peinado VI, Guitart M, Blanco I, Tura-Ceide O, Paul T, Barberà JA, Barreiro E. Am J Physiol Lung Cell Mol Physiol. 2023; 324(5): L677-L693. doi: 10.1152/ajplung.00258.2022. PMID: 36881560
Does arterial oxygenation during exercise add prognostic value in pulmonary arterial hypertension?
Authors: Alsina-Restoy X, Torres-Castro R, Torralba-García Y, Burgos F, Barberà JA, Agustí À, Blanco I. Respir Med. 2023 Jan;206:107070. doi: 10.1016/j.rmed.2022.107070. Epub 2022 Dec 5. PMID: 36508987
Oxidative Stress and Antioxidant Therapy in Pulmonary Hypertension.
Authors: Poyatos P, Gratacós M, Samuel K, Orriols R, Tura-Ceide O. Antioxidants (Basel). 2023 Apr 26;12(5):1006. doi: 10.3390/antiox12051006. PMID: 3723787
Editorial: Metabolic changes in vascular disorders: A path for early diagnosis and new therapeutic targets.
Authors: Ayyoub S, Blanco I, Izquierdo-García JL, Tura-Ceide O. Front Cardiovasc Med. 2023 Mar 3;10:1163298. doi: 10.3389/fcvm.2023.1163298. eCollection 2023. PMID: 36937922
Emerging Role of Balloon Pulmonary Angioplasty in Chronic Thromboembolic Pulmonary Disease-Insights from the 2022 ESC Guidelines.
Authors: Banaszkiewicz M, Kurzyna P, Kubikowska N, Mucha M, Rudnik A, Gąsecka A, Pietrasik A, Grabowski M, Jaguszewski MJ, Kasprzyk P, Kędzierski P, Ciećwierz D, Żuk G, Szwed P, Piłka M, Florczyk M, Kurzyna M, Darocha S.J Clin Med. 2023 Aug 16;12(16):5336. doi: 10.3390/jcm12165336.
Case report: Transected Hickman catheter and its thrombotic occlusion in a patient with idiopathic pulmonary arterial hypertension-can a catheter replacement be avoided?
Authors: Sławiński G, Zieleniewicz P, Faran A, Dąbrowska-Kugacka A, Kurzyna M, Kempa M, Daniłowicz-Szymanowicz L, Lewicka E.Front Cardiovasc Med. 2023 Jul 20;10:1230417. doi: 10.3389/fcvm.2023.1230417. eCollection 2023.PMID: 37547245
Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: a clinical consensus statement of the ESC working group on pulmonary circulation and right ventricular function.
Authors: Lang IM, Andreassen AK, Andersen A, Bouvaist H, Coghlan G, Escribano-Subias P, Jansa P, Kopec G, Kurzyna M, Matsubara H, Meyer BC, Palazzini M, Post MC, Pruszczyk P, Räber L, Roik M, Rosenkranz S, Wiedenroth CB, Redlin-Werle C, Brenot P. Eur Heart J. 2023 Aug 1;44(29):2659-2671. doi: 10.1093/eurheartj/ehad413. PMID: 37470202
Impact of diabetes mellitus on disease severity and patient survival in idiopathic pulmonary arterial hypertension: data from the Polish multicentre registry (BNP-PL).
Authors: Jonas K, Kurzyna M, Mroczek E, Chrzanowski Ł, Mularek-Kubzdela T, Skoczylas I, Błaszczak P, Grześk G, Mizia-Stec K, Kuśmierczyk B, Kamiński K, Lewicka E, Peregud-Pogorzelska M, Tomaszewski M, Jacheć W, Gąsior Z, Pawlak A, Ryczek R, Pruszczyk P, Doboszyńska A, Widejko-Pietkiewicz K, Zabłocka W, Waligóra M, Kopeć G. Cardiovasc Diabetol. 2023 Jul 1
Long COVID syndrome after SARS-CoV-2 survival in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.
Authors: Wieteska-Miłek M, Kuśmierczyk-Droszcz B, Betkier-Lipińska K, Szmit S, Florczyk M, Zieliński P, Hoffman P, Krzesińki P, Kurzyna M.Pulm Circ. 2023 May 31;13(2):e12244. doi: 10.1002/pul2.12244. eCollection 2023 Apr. PMID: 372661403;22(1):177. doi: 10.1186/s12933-023-01885-6.PMID: 37443009
Pulmonary arterial hypertension drugs can partially restore altered angiogenic capacities in bmpr2-silenced human lung microvascular endothelial cells. Authors: Tielemans, B., Wagenaar, A., Belge, C., Delcroix, M., Quarck, R. with Quarck, R. (corresp. author) (2023).. PULMONARY CIRCULATION, 13 (4), Art.No. ARTN e12293. doi: 10.1002/pul2.12293
Chronic thromboembolic pulmonary hypertension: realising the potential of multimodal management.
Authors: Delcroix, M., de Perrot, M., Jaïs, X., Jenkins, D.P., Lang, I.M., Matsubara, H., Meijboom, L.J., Quarck, R., Simonneau, G., Wiedenroth, C.B., Kim, N.H. (2023. Lancet Respir Med, 11 (9), 836-850. doi: 10.1016/S2213-2600(23)00292-8
Machine learning to differentiate pulmonary hypertension due to left heart disease from pulmonary arterial hypertension.
Authors: Swinnen, K., Verstraete, K., Baratto, C., Hardy, L., De Vos, M., Topalovic, M., Claessen, G., Quarck, R., Belge, C., Vachiery, J-L., Janssens, W., Delcroix, M. with Delcroix, M. (corresp. author) (2023). Machine learning to differentiate pulmonary hypertension due to left heart disease from pulmonary arterial hypertension. ERJ OPEN RESEARCH, 9 (5), Art.No. ARTN 229. doi: 10.1183/23120541.00229-2023
Medical and Interventional Treatment for Chronic Thromboembolic Pulmonary Hypertension.
Authors: Delcroix, M., Belge, C., Maleux, G., Godinas, L. with Delcroix, M. (corresp. author) (2023). Monographic Issue on Pulmonary Hypertension: SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE. doi: 10.1055/s-0043-1770122
Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: a COMPERA analysis.
Authors: Distler, O., Ofner, C., Huscher, D., Jordan, S., Ulrich, S., Staehler, G., Gruenig, E., Held, M., Ghofrani, H.A., Claussen, M., Lange, T.J., Klose, H., Rosenkranz, S., Vonk-Noordegraaf, A., Vizza, C.D., Delcroix, M., Opitz, C., Pausch, C., Scelsi, L., Neurohr, C., Olsson, K.M., Coghlan, J.G., Halank, M., Skowasch, D., Behr, J., Milger, K., Remppis, B.A., Skride, A., Jureviciene, E., Gumbiene, L., Miliauskas, S., Loeffler-Ragg, J., Wilkens, H., Pittrow, D., Hoeper, M.M., Ewert, R. with Distler, O. (corresp. author) (2023). RHEUMATOLOGY. doi: 10.1093/rheumatology/kead360
Novel acquisitions in chronic thromboembolic pulmonary hypertension.
Authors: Lang, I.M., Delcroix, M. with Lang, I.M. (corresp. author) (2023). EUROPEAN HEART JOURNAL, 44(27), 2417-2419. doi: 10.1093/eurheartj/ehad314
Impairment of Angiogenesis-Driven Clot Resolution Is a Key Event in the Progression to Chronic Thromboembolic Pulmonary Hypertension: Validation in a Novel Rabbit Model.
Authors: Quarck, R., Willems, L., Tielemans, B., Stoian, L., Ronisz, A., Wagenaar, A., Perros, F., Claessen, G., Ciarka, A., Godinas, L., Belge, C., Jacquemin, M., Delcroix, M. with Quarck, R. (corresp. author) (2023). ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY, 43(7), 1308-1321. doi: 10.1161/ATVBAHA.122.317262
Incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: an updated systematic review and meta-analysis.
Authors: Luijten, D., Talerico, R., Barco, S., Cannegieter, S.C., Delcroix, M., Ende-Verhaar, Y.M., Huisman, M.V., Konstantinidis, S., Mairuhu, A.T A., van Mens, T.E., Ninaber, M., Pruszczyk, P., Noordegraaf, A.V., Klok, F.A. with Klok, F.A. (corresp. author) (2023). EUROPEAN RESPIRATORY JOURNAL, 62 (1), Art.No. ARTN 2300449. doi: 10.1183/13993003.00449-2023
Anthracofibrosis mimicking chronic thromboembolic pulmonary hypertension.
Authors: Van Genechten, S., Meyns, B., Godinas, L., Maleux, G., Everaerts, S., Van Beersel, D., Belge, C., Weynand, B., Delcroix, M., Verbelen, T. with Verbelen, T. (corresp. author) (2023). PULMONARY CIRCULATION, 13(3), Art.No. ARTN e12263. doi: 10.1002/pul2.12263
The Cellular Landscape of Chronic Thromboembolic Pulmonary Hypertension Revealed by Single-Cell Sequencing: Therapeutic Implications?
Authors: Quarck, R., Bogaard, H.J., Delcroix, M. with Quarck, R. (corresp. author) (2023). AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 207(10), 1266-1268. doi: 10.1164/rccm.202302-0326ED
Right ventricular strain related to pulmonary artery pressure predicts clinical outcome in patients with pulmonary arterial hypertension.
Authors: Unlu, S., Bezy, S., Cvijic, M., Duchenne, J., Delcroix, M., Voigt, J.U. with Voigt, J.U. (corresp. author) (2023).. EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING, 24(5), 635-642. doi: 10.1093/ehjci/jeac136
European Society of Cardiology quality indicators for the care and outcomes of adults with pulmonary arterial hypertension. Developed in collaboration with the Heart Failure Association of the European Society of Cardiology.
Authors: Aktaa, S., Gale, C.P., Brida, M., Giannakoulas, G., Kovacs, G., Adir, Y., Benza, R.L., Boehm, M., Coats, A., D’Alto, M., Escribano-Subias, P., Ferrari, P., Galie, N., Gibbs, J.S R., Gin-Sing, W., Hoeper, M.M., Humbert, M., Lang, I.M., Maron, B.A., Meszaros, G., Noordegraaf, A.V., Price, L.C., Pepke-Zaba, J., Radegran, G., Reis, A., Sitbon, O., Torbicki, A., Ulrich, S., Rosenkranz, S., Delcroix, M. with Aktaa, S. (corresp. author) (2023). EUROPEAN JOURNAL OF HEART FAILURE, 25 (4), 469-477. doi: 10.1002/ejhf.2830
Highlights from the International Chronic Thromboembolic Pulmonary Hypertension Congress 2021.
Authors: Simonneau, G., Fadel, E., Noordegraaf, A.V., Toshner, M., Lang, I.M., Klok, F.A., McInnis, M.C., Screaton, N., Madani, M.M., Martinez, G., Salaunkey, K., Jenkins, D.P., Matsubara, H., Brenot, P., Hoeper, M.M., Ghofrani, H.A., Jais, X., Wiedenroth, C.B., Guth, S., Kim, N.H., Pepke-Zaba, J., Delcroix, M., Mayer, E. with Delcroix, M. (corresp. author) (2023). EUROPEAN RESPIRATORY REVIEW, 32(167), Art.No. ARTN 220132. doi: 10.1183/16000617.0132-2022
2022
Classification of COPD: fostering prevention and precision medicine in the Lancet Commission on COPD.
Authors: Brusselle GG, Humbert M. Lancet 2022 Sep 5 [Epub ahead of print]
The role of cardiopulmonary exercise testing in identifying and monitoring pulmonary veno-oclusive disease: a case report with ING.
Authors: Segura de la Cal T, Pérez-Olivares C, Cristo Ropero MJ, Luna López R, Escribano-Subías P.Eur Heart J Case Rep. 2022 Mar 28;6(5):ytac138. doi: 10.1093/ehjcr/ytac138. eCollection 2022 May.PMID: 35592750 Free PMC article.
Clinical Implications of the Genetic Background in Pediatric Pulmonary Arterial Hypertension: Data from the Spanish REHIPED Registry. Authors: Cruz-Utrilla A, Gallego-Zazo N, Tenorio-Castaño JA, Guillén I, Torrent-Vernetta A, Moya-Bonora A, Labrandero C, Rodríguez-Monte MEG, Rodríguez-Ogando A, Rey MDMRVD, Espín J, Plata-Izquierdo B, Álvarez-Fuente M, Moreno-Galdó A, Escribano-Subias P, Marín MJDC.Int J Mol Sci. 2022 Sep 9;23(18):10433. doi: 10.3390/ijms231810433.PMID: 36142358 Free PMC article.
Pulmonary thromboendarterectomy in chronic thromboembolic pulmonary hypertension: the Spanish experience.
Authors: María Jesús López-Gude, Isabel Blanco, Victoria Benito-Arnáiz, Manel Castellà, Pilar Escribano-Subías, Clara Martin, Joan Albert Barberà, José María Cortina-Romero. Ann Cardiothorac Surg. 2022 Mar;11(2):151-160. doi: 10.21037/acs-2021-pte-18.
PDE5 to keep them alive: The use of phosphodiesterase type-5 inhibitors in severe pulmonary hypertension associated with interstitial lung disease
Authors: Jason Weatherald, Marc Humbert. Respirology. 2022 Nov 1. doi: 10.1111/resp.14399. Online ahead of print.
Pulmonary thromboendarterectomy in chronic thromboembolic pulmonary hypertension: the Spanish experience.
Authors: María Jesús López-Gude, Isabel Blanco, Victoria Benito-Arnáiz, Manel Castellà, Pilar Escribano-Subías, Clara Martin, Joan Albert Barberà, José María Cortina-Romero. Ann Cardiothorac Surg. 2022 Mar;11(2):151-160. doi: 10.21037/acs-2021-pte-18.
Phenotypic diversity of vascular smooth muscle cells in pulmonary arterial hypertension: implications for therapy.
Authors: Lechartier B, Berrebeh N, Huertas A, Humbert M, Guignabert C, Tu L. Chest 2022; 161:219-31
Mendelian randomisation and experimental medicine approaches to IL-6 as a drug target in PAH.
Authors: Toshner M, Church C, Harbaum L, Rhodes C, Villar SS, Liley J, Jones R, Arora A, Batai K, Desai AA, Coghlan JG, Gibbs JSR, Gor D, Gräf S, Harlow L, Hernandez-Sanchez J, Howard LS, Humbert M, Karnes J, Kiely DG, Kittles R, Knightbridge E, Lam B, Lutz KA, Nichols WC, Pauciulo MW, Pepke-Zaba J, Suntharalingam J, Soubrier F, Trembath RC, Schwantes-An TL, Wort SJ, Wilkins M, Gaine S, Morrell NW, Corris PA. Eur Respir J 2022; 59:2002463.
Double-lung transplantation followed by delayed percutaneous repair for atrial septal defect-associated pulmonary arterial hypertension.
Authors: Gazengel P, Hascoët S, Amsallem M, Savale L, Montani D, Mercier O, Humbert M, Fadel E, Le Pavec J. Eur Respir J 2022; 59:2102388.
External validation of a refined 4-strata risk assessment score from the French pulmonary hypertension Registry.
Authors: Boucly A, Weatherald J, Savale L, de Groote P, Cottin V, Prévot G, Chaouat A, Picard F, Horeau-Langlard D, Bourdin A, Jutant EM, Beurnier A, Jevnikar M, Jaïs X, Simonneau G, Montani D, Sitbon O, Humbert M. Eur Respir J 2022; 59:2102419.
WASOG statement on the diagnosis and management of sarcoidosis-associated pulmonary hypertension. Authors: Savale L, Huitema M, Shlobin O, Kouranos V, Nathan SD, Nunes H, Gupta R, Grutters JC, Culver DA, Post MC, Ouellette D, Lower EE, Al-Hakim T, Wells AU, Humbert M, Baughman RP. Eur Respir Rev 2022; 31:210165.
Phenotypes of idiopathic pulmonary arterial hypertension
Authors: Alejandro Cruz-Utrilla1, Carmen Pérez-Olivares, Amaya Martínez-Meñaca Manuel López-Meseguer, Pilar Escribano-Subias Lancet Respir Med. 2022 Oct;10(10):e87.doi: 10.1016/S2213-2600(22)00290-9.
Pulmonary endarterectomy in patients with myeloproliferative neoplasms.
Authors: Genty T, Wirth C, Humbert M, Fadel E, Stéphan F. Chest 2022; 161:552-6.
Lung ventilation/perfusion scintigraphy for the screening of chronic thromboembolic pulmonary hypertension: which criteria to use?
Authors: Le Pennec R, Tromeur C, Orione C, Robin P, Le Mao R, De Moreuil C, Jevnikar M, Hoffman C, Savale L, Couturaud F, Sitbon O, Montani D, Jaïs X, Le Gal G, Salaün PY, Humbert M, Le Roux PY. Front Med (Lausanne) 2022; 9:851935.
Platelet-Derived Growth Factor receptor type α activation drives pulmonary vascular remodeling via progenitor cell proliferation and induces pulmonary hypertension.
Authors: Solinc J, Raimbault-Machado J, Dierick F, El Bernoussi L, Tu L, Thuillet R, Mougenot N, Hoareau-Coudert B, Monceau V, Pavoine C, Atassi F, Sassoon D, Marazzi G, Harvey RP, Schofield P, Christ D, Humbert M, Guignabert C, Soubrier F, Nadaud S. . J Am Heart Assoc 2022; 11:e023021.
Interplay of sex hormones and long-term right ventricular adaptation in a Dutch PAH-cohort. Authors: van Wezenbeek J, Groeneveldt JA, Llucià-Valldeperas A, van der Bruggen CE, Jansen SMA, Smits AJ, Smal R, van Leeuwen JW, Remedios CD, Keogh A, Humbert M, Dorfmüller P, Mercier O, Guignabert C, Niessen HWM, Handoko ML, Marcus JT, Meijboom LJ, Oosterveer FPT, Westerhof BE, Heijboer AC, Bogaard HJ, Noordegraaf AV, Goumans MJ, de Man FS. J Heart Lung Transplant 2022; 41:445-57.
[Hepatopulmonary syndrome: Prevalence, pathophysiology and clinical implications].
Authors: Certain MC, Robert F, Baron A, Sitbon O, Humbert M, Guignabert C, Tu L, Savale L. Rev Mal Respir 2022 ; 39 :84-9.
Aggressive afterload lowering to improve the RV: a new target for medical therapy in PAH?
Authors: Vizza CD, Lang IM, Badagliacca R, Benza RL, Rosenkranz S, White RJ, Adir Y, Andreassen AK, Balasubramanian V, Bartolome S, Blanco I, Bourge RC, Carlsen J, Conde Camacho RE, D’Alto M, Farber HW, Ford HJ, Frantz RP, Ghio S, Gomberg-Maitland M, Humbert M, Naeije R, Orfanos SE, Oudiz RJ, Perrone SV, Shlobin OA, Simon MA, Sitbon O, Torres F, Vachiery JL, Wang KY, Yacoub MH, Liu Y, Golden G, Matsubara H. Am J Respir Crit Care Med 2022; 205:751-60.
Pulmonary thromboendarterectomy: The Marie Lannelongue Hospital experience.
Authors: Mercier O, Dubost C, Delaporte A, Genty T, Fabre D, Mitilian D, Girault A, Issard J, Astaneh A, Menager JB, Dauriat G, Mussot S, Jevnikar M, Jais X, Humbert M, Simonneau G, Dartevelle P, Ion I, Stephan F, Brenot P, Fadel E.. Ann Cardiothorac Surg 2022; 11:143-50.
Chronic thromboembolic pulmonary hypertension: the magic of pathophysiology.
Authors: Simonneau G, Dorfmüller P, Guignabert C, Mercier O, Humbert M. Ann Cardiothorac Surg 2022; 11:106-19.
Finding pulmonary arterial hypertension: switching to offense to mitigate disease burden.
Authors: Maron BA, Humbert M. JAMA Cardiol 2022; 7:369-70
Diagnostic, prognostic and differential diagnostic relevance of pulmonary hemodynamics during exercise – a systematic review.
Authors: Zeder K, Banfi C, Steinrisser-Allex G, Maron BA, Humbert M, Lewis GD, Berghold A, Olschewski H, Kovacs G.. Eur Respir J 2022; 60:2103181.
Progression of pulmonary veno-occlusive disease without pulmonary hypertension.
Authors: Beshay S, Humbert M, Barrios R, Sahay S. Pulm Circ 2022; 12:e12046.
Sequential combination therapy with parenteral prostacyclin in BMPR2 mutations carriers.
Authors: Boucly A, Savale L, Jaïs X, Humbert M, Sitbon O, Montani D. Pulm Circ 2022; 12:e12023.
Pulsatile pulmonary artery pressure in a large animal model of chronic thromboembolic pulmonary hypertension: Similarities and differences with human data.
Authors: Boulate D, Loisel F, Coblence M, Provost B, Todesco A, Decante B, Beurnier A, Herve P, Perros F, Humbert M, Fadel E, Mercier O, Chemla D. Pulm Circ 2022; 12:e12017.
Different cardiovascular and pulmonary phenotypes for single- and double-knock-out mice deficient in BMP9 and BMP10.
Authors: Bouvard C, Tu L, Rossi M, Desroches-Castan A, Berrebeh N, Helfer E, Roelants C, Liu H, Ouarne M, Chaumontel N, Mallet C, Battail C, Bikfalvi A, Humbert M, Savale L, Daubon T, Perret P, Tillet E, Guignabert C, Bailly S. Cardiovasc Res 2022; 118:1805-20.
Using the plasma proteome for risk stratifying patients with pulmonary arterial hypertension.
Authors: Rhodes CJ, Wharton J, Swietlik EM, Harbaum L, Girerd B, Coghlan JG, Lordan J, Church C, Pepke-Zaba J, Toshner M, Wort SJ, Kiely DG, Condliffe R, Lawrie A, Gräf S, Montani D, Boucly A, Sitbon O, Humbert M, Howard LS, Morrell NW, Wilkins MR. Am J Respir Crit Care Med 2022; 205:1102-11.
Antigny F. SUR1 as a New Therapeutic Target for Pulmonary Arterial Hypertension.
Authors: Le Ribeuz H, Masson B, Capuano V, Dutheil M, Gooroochurn H, Boët A, Ghigna MR, De Montpreville V, Girerd B, Lambert M, Mercier O, Chung WK, Humbert M, Montani D, Am J Respir Cell Mol Biol 2022; 66:539-44.
Oral anticoagulants (NOAC and VKA) in chronic thromboembolic pulmonary hypertension.
Authors: Humbert M, Simonneau G, Pittrow D, Delcroix M, Pepke-Zaba J, Langleben D, Mielniczuk LM, Escribano Subias P, Snijder RJ, Barberà JA, Klotsche J, Meier C, Hoeper MM. J Heart Lung Transplant 2022; 41:716-21.
Mining the plasma proteome for insights into the molecular pathology of pulmonary arterial hypertension.
Authors: Harbaum L, Rhodes CJ, Wharton J, Lawrie A, Karnes JH, Desai AA, Nichols WC, Humbert M, Montani D, Girerd B, Sitbon O, Boehm M, Novoyatleva T, Schermuly RT, Ghofrani HA, Toshner M, Kiely DG, Howard LS, Swietlik EM, Gräf S, Pietzner M, Morrell NW, Wilkins MR. Am J Respir Crit Care Med 2022; 205:1449-60.
Erythrocytes are altered in pulmonary arterial hypertension.
Authors: Jutant EM, Tu L, Thuillet R, Picard V, Guignabert C, Parent F, Sitbon O, Humbert M, Savale L, Huertas A. Eur Respir J 2022; 59:2200506.
Severe pulmonary hypertension associated with COPD: long-term results of a prospective French multicentre cohort.
Authors: Dauriat G, Reynaud-Gaubert M, Cottin V, Lamia B, Montani D, Belhadi D, Humbert M, Laouenan C, Mal H. Eur Respir J 2022; 60:2102897.
An emerging phenotype of pulmonary arterial hypertension patients carrying SOX17 variants. Authors: Montani D, Lechartier B, Girerd B, Eyries M, Ghigna MR, Savale L, Jaïs X, Seferian A, Jevnikar M, Boucly A, Riou M, Traclet J, Chaouat A, Levy M, Le Pavec J, Fadel E, Perros F, Soubrier F, Remy-Jardin M, Sitbon O, Bonnet D, Humbert M. Eur Respir J 2022; 60:2200656.
Outcomes of cirrhotic patients with pre-capillary pulmonary hypertension and pulmonary vascular resistance between 2 and 3 Wood Units.
Authors: Certain MC, Baron A, Turpin M, Ebstein N, Boucly A, Beurnier A, Jevnikar M, Roche A, Keddache S, Bulifon S, Seferian A, Jaïs X, Montani D, Humbert M, Sitbon O, Savale L. Eur Respir J 2022; 60:2200107.
Screening for pulmonary veno-occlusive disease in heterozygous EIF2AK4 variant carriers.
Authors: Lechartier B, Girerd B, Eyries M, Beurnier A, Humbert M, Montani D. Eur Respir J 2022; 60:2200760.
Recent advances in the management of pulmonary hypertension with interstitial lung disease. Authors: Waxman AB, Elia D, Adir Y, Humbert M, Harari S. Eur Respir Rev 2022; 31:210220.
Loss of cAbl tyrosine kinase in pulmonary arterial hypertension causes dysfunction of vascular endothelial cells. Authors: Le Vely B, Phan C, Berrebeh N, Thuillet R, Ottaviani M, Chelgham MK, Chaumais MC, Amazit L, Humbert M, Huertas A, Guignabert C, Tu L. Am J Respir Cell Mol Biol 2022; 67:215-26.
COVID-19 in patients with pulmonary hypertension: a national prospective cohort study.
Authors: Montani D, Certain MC, Weatherald J, Jaïs X, Bulifon S, Noel-Savina E, Nieves A, Renard S, Traclet J, Bouvaist H, Riou M, de Groote P, Moceri P, Bertoletti L, Favrolt N, Guillaumot A, Jutant EM, Beurnier A, Boucly A, Ebstein N, Jevnikar M, Pichon J, Keddache S, Preda M, Roche A, Solinas S, Seferian A, Reynaud-Gaubert M, Cottin V, Savale L, Humbert M, Sitbon O Am J Respir Crit Care Med 2022 May 12 [Epub ahead of print]
Identifying new drugs associated with pulmonary arterial hypertension: A WHO pharmacovigilance database disproportionality analysis.
Authors: Hlavaty A, Roustit M, Montani D, Chaumais MC, Guignabert C, Humbert M, Cracowski JL, Khouri C. Br J Clin Pharmacol 2022 Jun 9 [Epub ahead of print]
Knowledge exchange between patient and pharmacist: a mixed methods study to explore the role of pharmacists in patient education and counselling in asthma and pulmonary arterial hypertension.
Authors: Renet S, Chaumais MC, Gallant-Dewavrin M, Jouet E, Bezie Y, Humbert M, Rieutord A, Vergnas OL. Ann Pharm Fr 2022 Jun 20 [Epub ahead of print]
Risk stratification in patients with pulmonary arterial hypertension at the time of listing for lung transplantation.
Authors: Vicaire H, Pavec JL, Mercier O, Montani D, Boucly A, Roche A, Pradère P, Dauriat G, Feuillet S, Pichon J, Jevnikar M, Beurnier A, Jaïs X, Fadel E, Sitbon O, Humbert M, Savale L. J Heart Lung Transplant 2022 Jun 6 [Epub ahead of print]
First genotype-phenotype study in TBX4 syndrome: gain-of-function mutations causative for lung disease.
Authors: Prapa M, Lago-Docampo M, Swietlik EM, Montani D, Eyries M, Humbert M, Welch CCL, Chung W, Berger RMF, Bogaard HJ, Danhaive O, Escribano-Subías P, Gall H, Girerd B, Hernandez-Gonzalez I, Holden S, Hunt D, Jansen SM, Kerstjens-Frederikse W, Kiely D, Lapunzina P, McDermott J, Moledina S, Pepke-Zaba J, Polwarth GJ, Schotte G, Tenorio-Castaño J, Thompson AAR, Wharton J, Wort SJ, Megy K, Mapeta R, Treacy CM, Martin JM, Li W, Swift AJ, Upton PD, Morrell NW, Gräf S, Valverde D. Am J Respir Crit Care Med 2022 Jul 19 [Epub ahead of print]
Pulmonary veno-occlusive disease associated with long-term occupational exposure to chemical solvents and pesticides.
Authors: Triantafyllidi H, Iordanidis D, Montani D, Samiotis E, Mademli M, Vlachos S, Mpahara A, Humbert M, Dorfmuller P, Tsagkaris I, Iliodromitis E.. A case report. Respir Med Res 2022 Jul 4 [Epub ahead of print]
Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (RACE): a multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up study.
Authors: Jaïs X, Brenot P, Bouvaist H, Jevnikar M, Canuet M, Chabanne C, Chaouat A, Cottin V, De Groote P, Favrolt N, Horeau-Langlard D, Magro P, Savale L, Prévot G, Renard S, Sitbon O, Parent F, Trésorier R, Tromeur C, Piedvache C, Grimaldi L, Fadel E, Montani D, Humbert M, Simonneau G. Lancet Respir Med 2022 Aug 1 [Epub ahead of print]
Multiparametric evaluation of right ventricular function in pulmonary arterial hypertension associated with congenital heart disease
Authors: Fournier E, Selegny M, Amsallem M, Haddad F, Cohen S, Valdeolmillos E, Le Pavec J, Humbert M, Isorni MA, Azarine A, Sitbon O, Jais X, Savale L, Montani D, Fadel E, Zoghbi J, Belli E, Hascoët S.. Rev Esp Cardiol (Engl Ed) 2022 Aug 5 [Epub ahead of print]
ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension.
Authors: Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, Carlsen J, Coats AJS, Escribano-Subias P, Ferrari P, Ferreira DS, Kiely DG, Mayer E, Meszaros G, Nagavci B, Olsson KM, Pepke-Zaba J, Quint JK, Radegran G, Simonneau G, Sitbon O, Tonia T, Toshner M, Vachiery JL, Vonk Noordegraaf A, Delcroix M, Rosenkranz S. Eur Heart J 2022 doi: 10.1093/eurheartj/ehac237
Sotatercept for the treatment of pulmonary arterial hypertension: PULSAR open-label extension.
Authors: Humbert M, McLaughlin V, Gibbs JSR, Gomberg-Maitland M, Hoeper MM, Preston IR, Souza R, Waxman A, Ghofrani HA, Escribano Subias P, Feldman J, Meyer G, Montani D, Olsson KM, Manimaran S, de Oliveira Pena J, Badesch DB. Eur Respir J 2022 Aug 30 [Epub ahead of print]
Mineralocorticoid receptor antagonism by finerenone attenuates established pulmonary hypertension in rats.
Authors: Tu L, Thuillet R, Perrot J, Ottaviani M, Ponsardin E, Kolkhof P, Humbert M, Viengchareun S, Lombès M, Guignabert C. Hypertension 2022 Aug 18 [Epub ahead of print]
Usefulness of genetics for clinical reclassification and refinement of prognostic stratification in pulmonary arterial hypertension.
Authors: Cruz-Utrilla A, Gallego-Zazo N, Pérez-Olivares C, Hernández-González I, Bedate P, Meñaca AM, Meseguer ML, Lapunzina P, Núñez MP, Parra NO, Valverde D, Tenorio-Castaño JA, Escribano-Subias P.Rev Esp Cardiol (Engl Ed). 2022 Nov 17:S1885-5857(22)00296-1. doi: 10.1016/j.rec.2022.11.002. Online ahead of print.PMID: 36403940 English, Spanish.
Lung transplantation for interstitial lung disease in idiopathic inflammatory myositis: a cohort study.
Authors: Rivière A, Picard C, Berastegui C, Mora VM, Bunel V, Godinas L, Salvaterra E, Rossetti V, Savale L, Israel-Biet D, Demant X, Bermudez J, Meloni F, Jaksch P, Magnusson J, Beaumont L, Perch M, Mornex JF, Knoop C, Aubert JD, Hervier B, Nunes H, Humbert M, Gottlieb J, Uzunhan Y, Le Pavec J. Am J Transplant 2022 Aug 21 [Epub ahead of print]
López-Gude MJ, Blanco I, Benito-Arnáiz V, Castellà M, Escribano-Subías P, Martin C, Barberà JA, Cortina-Romero JM.Ann Cardiothorac Surg. 2022 Mar;11(2):151-160. doi: 10.21037/acs-2021-pte-18.PMID: 35433371
Authors: Segura de la Cal T, Pérez-Olivares C, Cristo Ropero MJ, Luna López R, Escribano-Subías P.Eur Heart J Case Rep. 2022 Mar 28;6(5):ytac138. doi: 10.1093/ehjcr/ytac138. eCollection 2022 May.PMID: 35592750
2021
Involvement of CFTR in the pathogenesis of pulmonary arterial hypertension
Authors: Laurent Godinas, Keerthana Iyer, Gergely Meszaros, Rozenn Quarck, Pilar Escribano-Subias, Anton Vonk Noordegraaf, Pavel Jansa, Michele D’Alto, Milan Luknar, Senka Milutinov Ilic, Catharina Belge, Olivier Sitbon, Abílio Reis, Stephan Rosenkranz, Joanna Pepke-Zaba, Marc Humbert, Marion Delcroix. Orphanet J Rare Dis. 2021 May 1;16(1):196.doi: 10.1186/s13023-021-01752-1.
PH CARE COVID survey: an international patient survey on the care for pulmonary hypertension patients during the early phase of the COVID-19 pandemic.
Authors: Laurent Godinas, Keerthana Iyer, Gergely Meszaros, Rozenn Quarck, Pilar Escribano-Subias, Anton Vonk Noordegraaf, Pavel Jansa, Michele D’Alto, Milan Luknar, Senka Milutinov Ilic, Catharina Belge, Olivier Sitbon, Abílio Reis, Stephan Rosenkranz, Joanna Pepke-Zaba, Marc Humbert, Marion Delcroix. Orphanet J Rare Dis. 2021 May 1;16(1):196.doi: 10.1186/s13023-021-01752-1.
The Experience, Prerequisites, and the Barriers in Organizing a Specialized Rehabilitation Program for Patients with Pulmonary Hypertension.
Authors: Eglė Palevičiūtė, Lina Gumbienė, Elena Jurevičienė, Toma Šimbelytė , Ieva Laucevičienė, Aleksandras Laucevičius, Jūratė Barysienė, Christina A Eichstaedt, Nicola Benjamin, Ekkehard Grünig, Jelena Čelutkienė) Respiration. 2021;100(10):949-957.doi: 10.1159/000516331. Epub 2021 May 27.
The role of cardiopulmonary exercise test in identifying pulmonary veno-occlusive disease
Authors: Carmen Pérez-Olivares, Teresa Segura de la Cal, Ángela Flox-Camacho, Jorge Nuche, Jair Tenorio, Amaya Martínez Meñaca, Alejandro Cruz-Utrilla, Javier de la Cruz-Bertolo, Marte Pérez Núñez, Spanish Pah Consortium, Fernando Arribas-Ynsaurriaga, Pilar Escribano Subías). Eur Respir J. 2021 Jun 4;57(6):2100115. doi: 10.1183/13993003.00115-2021. Print 2021 Jun.
Radiological differences between chronic thromboembolic pulmonary disease (CTEPD) and chronic thromboembolic pulmonary hypertension (CTEPH).
Authors: Carmine Capone, Adele Valentini, Silvia Lina Spinillo, Catherine Klersy, Anna Celentano, Maurizio Pin, Cristian Monterosso, Roberto Dore, Emilio Maria Bassi, Michela Zacchino, Giuseppe Rodolico, Angelo Guido Corsico, Lorenzo Preda, Stefano Ghio, Andrea Maria D’Armini. Eur Radiol. 2021 Aug;31(8):6230-6238.doi: 10.1007/s00330-020-07556-4. Epub 2021 Jan 28.
Looking forward: key initiatives to improve the care of rare diseases and streamline the delivery of medicines and vaccines in Europe.
Authors: Marc Humbert, Anita K Simonds Am J Physiol Lung Cell Mol Physiol. 2021 Sep 1;321(3):L616-L618. doi: 10.1152/ajplung.00317.2021. Epub 2021 Jul 28.
Radiological differences between chronic thromboembolic pulmonary disease (CTEPD) and chronic thromboembolic pulmonary hypertension (CTEPH).
Authors: Capone C, Valentini A, Spinillo SL, Klersy C, Celentano A, Pin M, Monterosso C, Dore R, Bassi EM, Zacchino M, Rodolico G, Corsico AG, Preda L, Ghio S, D’Armini AM
Radiological Findings in Multidetector Computed Tomography (MDCT) of Hereditary and Sporadic Pulmonary Veno-Occlusive Disease: Certainties and Uncertainties.
Authors: Pérez Núñez M, Alonso Charterina S, Pérez-Olivares C, Revilla Ostolaza Y, Morales Ruiz R, Enguita Valls AB, Tenorio JA, Gallego Zazo N, De Pablo Gafas A, Lapunzina P, Rodríguez Chaverri A, Escribano Subías P.
Pulmonary Hypertension in Patients With COPD: Results From the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA)
Authors: Carmine Dario Vizza, Marius M Hoeper, Doerte Huscher, David Pittrow, Nicola Benjamin, Karen M Olsson, H Ardeschir Ghofrani, Matthias Held, Hans Klose, Tobias Lange, Stephan Rosenkranz, Daniel Dumitrescu, Roberto Badagliacca, Martin Claussen, Michael Halank, Anton Vonk-Noordegraaf, Dirk Skowasch, Ralf Ewert, J Simon R Gibbs, Marion Delcroix, Andris Skride, Gerry Coghlan, Silvia Ulrich, Christian Opitz, Harald Kaemmerer, Oliver Distler, Ekkehard Grünig
Switching to riociguat versus maintenance therapy with phosphodiesterase-5 inhibitors in patients with pulmonary arterial hypertension (REPLACE): a multicentre, open-label, randomised controlled trial
Authors: Marius M Hoeper, Hikmet Al-Hiti, Raymond L Benza, Sung-A Chang, Paul A Corris, J Simon R Gibbs, Ekkehard Grünig, Pavel Jansa, James R Klinger, David Langleben, Vallerie V McLaughlin, Gisela M B Meyer, Jaquelina Ota-Arakaki, Andrew J Peacock, Tomás Pulido, Stephan Rosenkranz, Carmine Dario Vizza, Anton Vonk-Noordegraaf, R James White, Mikyung Chang, Frank Kleinjung, Christian Meier, Karen Paraschin, Hossein Ardeschir Ghofrani, Gérald Simonneau, REPLACE investigators
2020
ERS Statement on Chronic Thromboembolic Pulmonary Hypertension.
Authors: Delcroix M, Torbicki A, Gopalan D, Sitbon O, Klok FA, Lang I, Jenkins D, Kim NH, Humbert M, Jais X, Noordegraaf AV, Pepke-Zaba J, Brénot P, Dorfmuller P, Fadel E, Ghofrani HA, Hoeper MM, Jansa P, Madani M, Matsubara H, Ogo T, Grünig E, D’Armini A, Galie N, Meyer B, Corkery P, Meszaros G, Mayer E, Simonneau G.
Hemodynamic response to treatment and outcome in pulmonary hypertension associated with interstitial lung disease versus pulmonary arterial hypertension in systemic sclerosis.
Authors: Chauvelot L, Gamondes D, Berthiller J, Nieves A, Renard S, Catella-Chatron J, Ahmad K, Bertoletti L, Camara B, Gomez E, Launay D, Montani D, Mornex JF, Prévot G, Sanchez O, Schott AM, Subtil F, Traclet J, Turquier S, Zeghmar S, Habib G, Reynaud-Gaubert M, Humbert M, Cottin V.
Prediction of Prognostic Hemodynamic Indices in Pulmonary Hypertension Using Non-Invasive Parameters
Authors: Mańczak R, Kurzyna M, Piłka M, Darocha S, Florczyk M, Wieteska-Miłek M, Mańczak M, Torbicki A
Feasibility of a Noninvasive Operability Assessment in Chronic Thromboembolic Pulmonary Hypertension under Real-World Practice
Authors: Rodriguez Chaverri A, Revilla Ostolaza Y, Lopez-Gude MJ, Velazquez MT, Ponz de Antonio I, Alonso Charterina S, Albarran Gonzalez-Trevilla A, Perez Nunez M, Perez Vela JL, Morales Ruiz R, Delgado Jimenez JF, Arribas Ynsaurriaga F, Cortina JM, Escribano Subias P
2019
Precision medicine: The future of diagnostic approach to pulmonary hypertension?
Authors: Kedzierski P, Torbicki A
Highlights from the ERS International Congress 2018: Assembly 13 – Pulmonary Vascular Diseases.
Authors: Ramjug S, Adão R, Lewis R, Coste F, de Man F, Jimenez D, Sitbon O, Delcroix M, Vonk-Noordegraaf A.
2026
ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease developed by the task force for connective tissue disease-associated interstitial lung disease of the European Respiratory Society (ERS) and the European Alliance of Associations for Rheumatology (EULAR) Endorsed by the European Reference Network on rare respiratory diseases (ERN–LUNG).
Authors: Antoniou KM, Distler O, Gheorghiu AM, Moor CC, Vikse J, Bizymi N, Galetti I, Brown G, Bargagli E, Allanore Y, Corte TJ, Dieudé P, Cottin V, Fisher BA, Fabre A, Giles JT, Kreuter M, Lundberg IE, Poletti V, Maurer B, Renzoni EA, Müller-Ladner U, Strek ME, Sverzellati N, Studenic P, Mohammed J, Nagavci B, Stamm T, Tonia T, Crestani B, Hoffmann-Vold AM.Ann Rheum Dis. 2026 Jan;85(1):22-60. doi: 10.1016/j.ard.2025.08.021. Epub 2025 Sep 4.PMID: 40912974 Free article.
International validation of electronic nose technology as a diagnostic tool for fibrotic interstitial lung diseases.
Authors: Formsma BJ, Van Der Sar IG, Yazbeck L, Kreuter M, Cottin V, Polke M, Corte TJ, Tran M, Molyneaux PL, Moor CC, Wijsenbeek MS.Am J Respir Crit Care Med. 2026 May 1;212(5):981-988. doi: 10.1093/ajrccm/aamaf085.PMID: 41738130 Free article.
Design of a phase II randomised, double-blind, placebo-controlled, dose-finding trial of BI 1819479 in patients with idiopathic pulmonary fibrosis.
Authors: Wuyts WA, Bonella F, Ishii H, Lee JS, Renzoni E, Hadl S, Krzykalla J, Stowasser S, Engel M, Molina MM.ERJ Open Res. 2026 Mar 16;12(2):00973-2025. doi: 10.1183/23120541.00973-2025. eCollection 2026 Mar.PMID: 41846682 Free PMC article
Design of PROGRESSION-IPF: A pragmatic, open-label, randomized trial of patients with progressive disease in idiopathic pulmonary fibrosis.
Authors: Cottin V, Crestani B, Planès C, Chambellan A, Charloux A, Degano B, Gille T, Glérant JC, Jouneau S, Mewton N, Nunes H, Perez T, Samson G, Traclet J, Turquier S, Varillon Y, Wemeau-Stervinou L, Yavordios S, Amaz C; OrphaLung network.Respir Med Res. 2026 May;89:101266. doi: 10.1016/j.resmer.2026.101266. Epub 2026 Mar 27.PMID: 42025256 Free article. Clinical Trial.
Artificial intelligence for interstitial lung disease pattern recognition on high-resolution computed tomography: A step forward for general radiologists.
Authors: Pozzessere C, Si-Mohamed SA.Diagn Interv Imaging. 2026 Jul 28:S2211-5684(26)00131-2. doi: 10.1016/j.diii.2026.07.004. Online ahead of print.PMID: 42521552 No abstract available.
CT features of interstitial lung disease in systemic lupus erythematosus and overlap lupus-other connective tissue disease.
Authors: Sajust de Bergues de Escalup A, Mageau A, Deneuville L, Sacre K, Khalil A, Costedoat-Chalumeau N, Hachulla E, Uzunhan Y, Le Tallec E, Cadranel J, Marchand-Adam S, Montani D, Reynaud-Gaubert M, Prévot G, Beltramo G, Crestani B, Cottin V, Borie R, Debray MP; OrphaLung Network.Eur Radiol. 2026 May;36(5):4074-4085. doi: 10.1007/s00330-025-12106-x. Epub 2025 Nov 19.PMID: 41261180
Combination Antifibrotic Therapy in IPF/PPF: Learnings from Real-World Data.
Authors: Cottin V, Martínez Besteiro E, Meersseman C.Arch Bronconeumol. 2026 May;62(5):342-343. doi: 10.1016/j.arbres.2026.03.007. Epub 2026 Mar 25.PMID: 42083822 English, Spanish. No abstract available.
Autoantigen identification in antinuclear antibody-positive usual interstitial pneumonia without known autoantibody through immunoprecipitation-mass spectrometry.
Authors: Vulsteke JB, De Langhe E, De Sadeleer L, De Crem N, Yserbyt J, Dubbeldam A, Wuyts WA, Bossuyt X.ERJ Open Res. 2026 May 26;12(3):01156-2025. doi: 10.1183/23120541.01156-2025. eCollection 2026 May.PMID: 42206017 Free PMC article.
“Good health” and care needs in idiopathic pulmonary fibrosis/progressive pulmonary fibrosis: the COCOS-IPF patient-led European focus groups.
Authors: Schenk S, Boyd J, Dobbels F, Delameillieure A, Breuls S, Somogyi V, Van Bulck L, Sen Bircan E, Toreyin N, Magafa E, Vogiatzaki E, Solheim J, Straede B, Simaels P, Galvin L, Tonia T, Mogulkoc N, Kreuter M, Wuyts WA, Jones S, Stenzel N.ERJ Open Res. 2026 Jul 13;12(4):01361-2025. doi: 10.1183/23120541.01361-2025. eCollection 2026 Jul.PMID: 42445231 Free PMC article.
Prognostic factors in interstitial lung disease-associated pulmonary hypertension: data from the HYPID cohort and the French Pulmonary Hypertension Registry.
Authors: Diesler R, Turquier S, Reynaud-Gaubert M, Lestelle F, Lacoste-Palasset T, Valentin V, Quétant S, Chaouat A, Boissin C, Noël-Savina É, Tromeur C, Justet A, Maurac A, Artaud-Macari É, Nunes H, Bertoletti L, Magro P, Horeau-Langlard D, Seronde MF, Favrolt N, Trésorier R, Lamia B, Chabanne C, Riou M, Renard S, Sanchez O, Gagnadoux F, Nieves A, Ahmad K, Traclet J, Sitbon O, Lamblin N, Degano B, Valentin S, Bourdin A, Humbert M, Boucly A, Subtil F, Montani D, Cottin V; PulmoTension and OrphaLung networks.Eur Respir J. 2026 Mar 26:2502233. doi: 10.1183/13993003.02233-2025. Online ahead of print.PMID: 41887671
Prevalence, detection, and trajectory of combined pulmonary fibrosis and emphysema.
Authors: Khor YH, Marinescu DC, Manganas H, Morisset J, Muller NL, Hague CJ, Murphy D, Churg A, Wright JL, Al-Arnawoot A, Bilawich AM, Bourgouin P, Cox G, Elliot T, Ellis J, Fisher JH, Fladeland D, Grant-Orser A, Goobie GC, Guenther Z, Haider E, Hambly N, Huynh J, Johannson KA, Karjala G, Khalil N, Kolb M, Leipsic J, Lok S, MacIsaac S, McInnis M, Marcoux V, Mayo J, Scallan C, Sedlic T, Shapera S, Sun K, Tan V, Wong AW, Zheng B, Cottin V, Ryerson CJ.Chest. 2026 Jul 7:S0012-3692(26)00952-9. doi: 10.1016/j.chest.2026.05.051. Online ahead of print.PMID: 42413722 Free article.
Aspergillosis complicating idiopathic lung fibrosis: a multicentric series.
Authors: Bermudez J, Debray MP, Uzunhan Y, Marchand-Adam S, Nunes H, Godet C, Maitre T, Claustre J, Milesi J, Nasser M, Cottin V, Reynaud-Gaubert M, Crestani B.Respir Med. 2026 Jul;258:108885. doi: 10.1016/j.rmed.2026.108885. Epub 2026 May 12.PMID: 42128186 Free article.
Simultaneous pulmonary ventilation and perfusion imaging using spectral photon counting CT with xenon and gadolinium-based agents in combination with color K-edge imaging.
Authors: Robert A, Poletto S, Erhard K, Antonuccio MN, Hernandez CA, Gutwinska A, Coulibaly RS, Davaux F, Langlois JB, Boussel L, Douek P, Cottin V, Rit S, Dellacà RL, Bayat S, Si-Mohamed SA.Diagn Interv Imaging. 2026 Jun 24:S2211-5684(26)00108-7. doi: 10.1016/j.diii.2026.06.003. Online ahead of print.PMID: 42342552 Free article.
Behavioural changes in exposure patterns after genetic counselling among asymptomatic first-degree relatives of patients with pulmonary fibrosis and carriers of a telomere-related gene variant.
Authors: Sesé L, Bouvry D, Kannengiesser C, Wémeau-Stervinou L, Jouneau S, Prevot G, Vagnarelli S, Guérin C, Lassus A, Crestani B, Bonniaud P, Cottin V, Gueguen S, Nunes H, Borie R.ERJ Open Res. 2026 Mar 30;12(2):01396-2025. doi: 10.1183/23120541.01396-2025. eCollection 2026 Mar.PMID: 41918950 Free PMC article.
Adult male patients with DKC1 mutations present early-onset pulmonary fibrosis and severe prognosis.
Authors: Evrard O, Philippot Q, Kannengiesser C, Debray MP, Guyard A, Fattori A, Schuller A, Nunes H, Montani D, Bours V, Cottin V, Traclet J, Benattia A, Berthoux E, Daniel A, Yan-Min X, Lainey E, Saussereau J, Chelbi Viallon M, Oudin C, Morel H, Crestani B, Borie R, Ba I.ERJ Open Res. 2026 Jan 19;12(1):00417-2025. doi: 10.1183/23120541.00417-2025. eCollection 2026 Jan.PMID: 41561101 Free PMC article.
Expert Perspectives: Defining and Managing Progressive Pulmonary Fibrosis in Systemic Sclerosis.
Authors: Benfaremo D, Khanna D, Campochiaro C, Cottin V, Luppi F, Matucci-Cerinic M, Moroncini G, Allanore Y.Arthritis Rheumatol. 2026 Jun 1. doi: 10.1002/art.70237. Online ahead of print.PMID: 42223210 Review.
Clinical characteristics of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.
Authors: O’Callaghan M, Forde SH, Franciosi AN, Penugonda M, Diesler R, O’Brien H, Buikhuisen W, Almeamar H, Samhouri BF, Ryu JH, Veltkamp M, Manali ED, Lazaratou A, Papiris SA, Bonella F, Carr L, Cottin V, Taille C, McCormack FX, Gupta N, Strosberg J, Lococo FM, Harari S, Pelosi G, Papa R, Bouros D, Kolilekas L, Daniil Z, Dimeas I, Hernandez-Gonzalez F, Sellares J, Spagnolo P, Crowley RK, O’ Toole D, O’Shea D, Quinn S, Murphy DJ, Fabre A, Byrne AJ, Keane MP, Fournel L, McCarthy C.ERJ Open Res. 2026 Jan 19;12(1):00829-2025. doi: 10.1183/23120541.00829-2025. eCollection 2026 Jan.PMID: 41561106 Free PMC article.
Prognostic biomarkers for idiopathic pulmonary fibrosis: findings from ISABELA clinical trials.
Authors: Randall MJ, Andersen CA, Brown KK, de Bernard S, Ford P, Kaminski N, Kreuter M, Lim S, Maher TM, Prasad N, Prasse A, Pujuguet P, Teneggi V, van den Blink B, Wain LV, Watkins TR, Wuyts W, Bauer Y.ERJ Open Res. 2026 Feb 16;12(1):00893-2025. doi: 10.1183/23120541.00893-2025. eCollection 2026 Jan.PMID: 41704719 Free PMC article.
Real-world persistence with antifibrotic treatments: a nationwide study on claims data in France (The REPEAT study).
Authors: Cottin V, Najean M, Arnee E, Voulgaridou M, Dillard C, Ansolabehere X, Doublet M, Chollet J, Leroy S, Jouneau S.Respir Res. 2026 Jun 19. doi: 10.1186/s12931-026-03754-8. Online ahead of print.PMID: 42316168 Free article.
Molecular Investigation in Early-Onset Interstitial Lung Disease: Results From 699 Unrelated Patients.
Authors: Louvrier C, Nathan N, Cottin V, Desroziers T, Nau V, Soreze Y, Dastot-Le Moal F, Reix P, Bouvry D, Thumerelle C, Reynaud-Gaubert M, Hadchouel A, Prévot G, Manali E, Kannengiesser C, Ba I, Amselem S, Houdouin V, Borie R, Legendre M; RespiRare and OrphaLung.Respirology. 2026 Jan;31(1):53-61. doi: 10.1111/resp.70132. Epub 2025 Oct 3.PMID: 41041870 Free PMC article.
Effect of nerandomilast on survival in patients with pulmonary fibrosis.
Authors: Oldham JM, Assassi S, Azuma A, Cottin V, Hoffmann-Vold AM, Kreuter M, Maher TM, Richeldi L, Valenzuela C, Wijsenbeek MS, Choy S, Gu H, Weimann G, Ritter I, Stowasser S, Zoz DF, Martinez FJ.Eur Respir J. 2026 Jul 30:2600338. doi: 10.1183/13993003.00338-2026. Online ahead of print.PMID: 42532543
Efficacy and safety of danazol for pulmonary fibrosis or bone-marrow failure associated with telomere-related gene mutation.
Authors: Sicre de Fontbrune F, Jouneau S, Cottin V, Prevot G, Nunes H, Terriou L, Marchand-Adam S, Crestani B, Marcais A, Bergeron A, Debray MP, Plessier A, Lainey E, Ba I, Kannengiesser C, Desseaux K, Resche-Rigon M, Biard L, Borie R.ERJ Open Res. 2026 Apr 7;12(2):00905-2025. doi: 10.1183/23120541.00905-2025. eCollection 2026 Mar.PMID: 41953763 Free PMC article.
Acute exacerbation in fibrotic interstitial lung disease: An International Working Group Report.
Authors: Khor YH, Luppi F, Adegunsoye A, Collins BF, Farrand E, Montesi SB, Newton CA, Cottin V, Johannson KA, Kaul B, Kolb M, Kreuter M, Molyneaux PL, Wijsenbeek MS, Antoniou K, Behr J, Bendstrup E, Collard HR, Corte TJ, Drake WP, Ferrara G, Hariri LP, Hogaboam CM, Jenkins RG, Kaminski N, Kazerooni EA, Keane MP, Kondoh Y, Lee JS, Luo F, Maher TM, Martinez FJ, Moodley Y, Richeldi L, Sebastiani M, Sime PJ, Stowasser S, Tomassetti S, Wells A, Ryerson CJ, Podolanczuk AJ.Am J Respir Crit Care Med. 2026 Jul 14:aamag363. doi: 10.1093/ajrccm/aamag363. Online ahead of print.PMID: 42447235 Free article.
A statistical model for lung function trajectory and mortality in patients with fibrotic interstitial lung disease.
Authors: Wendelberger B, Jensen TP, Quintana M, Stewart I, Molyneaux PL, Maher TM, Oldham JM, Johnson SR, Fahy WA, Khan F, Saini G, Abdulqawi R, Allen R, Baldi BG, Chaudhuri N, Corte TJ, Cottin V, Funke-Chambour M, Glaspole I, Holland AE, Johannson KA, Khor YH, Kreuter M, Kulkarni T, Molina-Molina M, Martinez FJ, Montesi SB, Nathan SD, Piccari L, Rajan S, Rivera-Ortega P, Ryerson CJ, Sakamoto K, Wain LV, Wells AU, Adams W, Kawano-Dourado L, Jenkins RG, Lewis RJ; REMAP-ILD Consortium Investigators.Am J Respir Crit Care Med. 2026 Jul 1;212(7):1533-1547. doi: 10.1093/ajrccm/aamag103.PMID: 42085272
Bronchiolocentric interstitial pneumonia: words matter.
Authors: Cottin V.Am J Respir Crit Care Med. 2026 Mar 1;212(3):548-550. doi: 10.1093/ajrccm/aamag010.PMID: 41738195 Free article. No abstract available.
ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease.
Authors: Antoniou KM, Distler O, Gheorghiu AM, Moor CC, Vikse J, Bizymi N, Galetti I, Brown G, Bargagli E, Allanore Y, Corte TJ, Dieudé P, Cottin V, Fisher BA, Fabre A, Giles JT, Kreuter M, Lundberg IE, Poletti V, Maurer B, Renzoni EA, Müller-Ladner U, Strek ME, Sverzellati N, Studenic P, Mohammed J, Nagavci B, Stamm T, Tonia T, Crestani B, Hoffmann-Vold AM.Eur Respir J. 2026 Jan 22;67(1):2402533. doi: 10.1183/13993003.02533-2024. Print 2026 Jan.PMID: 40907995 Free PMC article
Phase 3 Trials of Inhaled Treprostinil for Idiopathic Pulmonary Fibrosis.
Authors: Nathan SD, Smith P, Deng C, King CS, De Salvo M, Weigt SS, Pandya S, Bandyopadhyay D, Lasky JA, Krishna R, Hambly N, Ramaswamy M, Kebbe J, Ettinger N, Gao J, Rao Y, Breytenbach N, Peterson L, Bell H, Behr J, Cottin V, Flaherty KR; TETON-1 Trial Investigators.N Engl J Med. 2026 Jul 9;395(2):115-126. doi: 10.1056/NEJMoa2501488. Epub 2026 May 18.PMID: 42149993 Clinical Trials
Nerandomilast in progressive pulmonary fibrosis: data from the whole follow-up period of the FIBRONEER-ILD trial.
Authors: Wijsenbeek MS, Assassi S, Azuma A, Cottin V, Hoffmann-Vold AM, Maher TM, Martinez FJ, Oldham JM, Richeldi L, Valenzuela C, Gu H, Ritter I, Stowasser S, Kreuter M; FIBRONEER-ILD Trial Investigators.Eur Respir J. 2026 Jul 9;68(1):2501899. doi: 10.1183/13993003.01899-2025. Print 2026 Jul.PMID: 41887669 Free PMC article. Clinical Trial.
Nerandomilast in idiopathic pulmonary fibrosis: data from the whole follow-up period of the FIBRONEER-IPF trial.
Authors: Oldham JM, Azuma A, Kreuter M, Maher TM, Martinez FJ, Richeldi L, Valenzuela C, Wijsenbeek MS, Liu Y, Zisa D, Zoz DF, Cottin V; FIBRONEER-IPF trial investigators.Am J Respir Crit Care Med. 2026 May 1;212(5):972-980. doi: 10.1093/ajrccm/aamag058.PMID: 41738262 Free article. Clinical Trial.
Nintedanib Combined With Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis or Progressive Pulmonary Fibrosis: A Long-Term Retrospective Multicentre Study (Combi-PF).
Authors: Meersseman C, Martínez Besteiro E, Romain-Scelle N, Crestani B, Marchand-Adam S, Nunes H, Wémeau-Stervinou L, Borie R, Diesler R, Valenzuela C, Cottin V; OrphaLung network.Arch Bronconeumol. 2026 May;62(5):301-309. doi: 10.1016/j.arbres.2025.10.004. Epub 2025 Oct 10.PMID: 41152077 Free article. English, Spanish.
High-Throughput Proteomics in Lymphangioleiomyomatosis: Premelanosome Protein as a Diagnostic Biomarker, Construction of a Diagnosis Score, and Evidence of Neutrophil Involvement.
Authors: Diesler R, Han T, Steagall WK, Salem S, Khabibullin D, Chami J, Squitieri B, Jones AM, Julien-Williams P, Machado T, Worthy T, Pacheco-Rodriguez G, Tang Y, Leroux C, Cottin V, Liu HJ, El-Chemaly S, McCormack FX, Moss J, Henske EP.Chest. 2026 Jun;169(6):1599-1615. doi: 10.1016/j.chest.2025.12.026. Epub 2025 Dec 31.PMID: 41482115 Free PMC article
Lung disease in rheumatoid arthritis.
Authors: Bendstrup E, Dieude P, Kreuter M, Hoffmann-Vold AM, Cottin V, Hauge EM.Nat Rev Rheumatol. 2026 Jun 18. doi: 10.1038/s41584-026-01394-2. Online ahead of print.PMID: 42315602 Review.
Clinical utility of lung biopsy in fibrotic interstitial lung disease.
Authors: Mor E, Piciucchi S, Ravaglia C, Aronson K, Poletti V, Hariri LP, Khor YH, Churg A, Collins B, Fabre A, Adegunsoye A, Chung JH, Cooper WA, Cottin V, Dotan Y, Hague C, Inoue Y, Jacob J, Martinez F, Podolanczuk AJ, Tomassetti S, Wells AU, Wijsenbeek MS, Yasufuku K, Dominy L, Mcdonald J, Sangari S, Ryerson CJ.Am J Respir Crit Care Med. 2026 Mar 1;212(3):571-590. doi: 10.1093/ajrccm/aamaf123.PMID: 41738223 Review.
Inhaled Treprostinil for Idiopathic Pulmonary Fibrosis.
Authors: Nathan SD, Smith P, Deng C, De Salvo M, Wuyts W, Pavie-Gallegos J, Song JW, Kramer MR, King CS, Mackintosh JA, Chambers D, Miranda GV, Breytenbach N, Peterson L, Bell H, Flaherty KR, Behr J, Cottin V; TETON-2 Trial Investigators.N Engl J Med. 2026 Jul 9;395(2):127-137. doi: 10.1056/NEJMoa2512911. Epub 2026 Mar 11.PMID: 41812190 Clinical Trial.
Progressive pulmonary fibrosis: a state-of-the-art review.
Authors: Cottin V, Brown K, Flaherty KR, Kolb M, Lynch DA, Oldham J, Valenzuela C, Wijsenbeek M, Wells AU.Eur Respir J. 2026 Jun 11:2501557. doi: 10.1183/13993003.01557-2025. Online ahead of print.PMID: 42276742 Review.
Efficacy and safety of nerandomilast in patients with autoimmune disease-related progressive pulmonary fibrosis in the FIBRONEER-ILD trial.
Authors: Hoffmann-Vold AM, Assassi S, Cottin V, Kreuter M, Valenzuela C, Wijsenbeek MS, Gu H, Kanakapura M, Ritter I, Stowasser S, Weimann G, Maher TM.Ann Rheum Dis. 2026 Apr 29:S0003-4967(26)00214-1. doi: 10.1016/j.ard.2026.03.027. Online ahead of print.PMID: 42062162 Free article.
2025
Thoracic Ultrasound applicability in the assessment of patients with fibrosing interstitial lung diseases: a systematic review.
Authors: Patabendige S, Falster C, Langkilde HZ, Harders SM, Bendstrup E, Durheim MT, Davidsen JRR. Ultraschall Med. 2026 Jan 12. doi: 10.1055/a-2785-1857.
The prevalence of interstitial lung disease and bronchiectasis in RA. A systematic review and meta-analysis.
Authors: Bækdahl JA, Janns LK, Hansen ESH, Meteran H, Bendstrup E, Ulrik CS. Sarc Vasc Diff Lung Dis 2025; 42 (4): 17112.
Access to Lymphangioleiomyomatosis Care Across Europe: An ERN-LUNG Survey of Clinical Practice and the Case for a European Registry and Trial Network.
Authors: O’Malley D, Humbert-Dorfmüller E, Barberà JA, van Batenburg A, Bendstrup E, Cottin V, Elia D, Froidure A, Grutters J, Harari S, Hernandez-Gonzalez F, Kokosi M, Lund TK, Manali ED, Meszaros G, van Pottelberge G, Radzikowska E, Sellares J, Svenningsson A, Veltkamp M, Wagner T, Hebestreit H, McCarthy C. ERJ Open Res 2025; in press (https://doi.org/10.1183/23120541.01010-2025).
Pulmonary alveolar proteinosis in Denmark: a retrospective cohort study.
Authors: Rønnov-Jessen I, Bendstrup E. Eur Clin Respir J 2025; 30;12(1):2597123.
Healthcare utilization preceding the diagnosis of interstitial lung disease.
Authors: Prior RS. Vestergaard CH, Kronborg-White S, Bendstrup E, Prior A. ERJ Open Res 2025.
Patient reported respiratory symptoms and lung radiation doses 11 years after loco-regional breast cancer radiation therapy in the DBCG RT Nation Study.
Authors: Sharma MB, Refsgaard LH, Bendstrup E, Buhl ES, Zachariae R, Friis RB, Farver-Vestergaard I, Korreman SS, Offersen BV Acta Oncol. 2025 Oct 1;64:1342-1350.
Pulmonary alveolar microlithiasis: A disorder of pulmonary phosphate homeostasis.
Authors: Zhou CY, Elwing JM, Bendstrup E, McCormack FX. Clinics in Chest Medicine 2025.
Phase 3 Trial of Inhaled Molgramostim in Autoimmune Pulmonary Alveolar Proteinosis.
Authors: Trapnell BC, Inoue Y, Bonella F, Wang T, McCarthy C, Arai T, Akasaka K, Mariani F, Mogulkoc N, Song JW, Baba T, Jouneau S, Numakura T, Ocal N, Mihaltan F, Ataya A, Bendstrup E, Campo I, B Carey, Arena R, Robinson B, Fleming R, Wasfi Y, Pratt R. N Engl J Med 2025; 393: 764-73.
Recent advances in the diagnosis and management of pulmonary alveolar proteinosis.
Authors: Bendstrup E, Lynn E, O’Callaghan M. Expert Rev Respir Med. 2025.
Angiotensin-converting enzyme genotypes and sarcoidosis: Correlation with susceptibility, progression and treatment response.
Authors: Møller J, Hilberg O, Nissen PH, Møller HJ, Bendstrup E. Sarcoidosis Vasc Diffuse Lung Dis. 2025; 42(2): 16324.
Basement membrane repair response biomarker PRO-C4 predicts progression in idiopathic pulmonary fibrosis: analysis of the PFBIO and PROFILE cohorts.
Authors: Sand JMB, Frederiksen P, John AE, Simões FB, Hoyer N, Prior TS, AvdicBelltheus A, Molyneaux PL, Stewart ID, Fainberg HP, Johnson SR, Karsdal MA, Leeming DJ, Bendstrup E, Shaker SB, Maher TB, Jenkins G. Thorax 2025 Jun 19:thorax-2024-221868.
Using superDimension as navigation when performing cryobiopsies: a randomised-controlled study.
Authors: Kronborg-White S, Madsen LB, Rasmussen TR, Prior T, Helbo S, Møller J, Harders S, Tomassetti S, Poletti V, Bendstrup E. Respir. Med. 2025 Jun 18:108204.
Pulmonary function trajectories in rheumatoid arthritis-associated interstitial lung disease.
Authors: Hyldgaard C, Pedersen AR, Ellingsen T, Bendstrup E. Scand. J. Rheumatol. 2025; 00: 1–7
Comparing the efficacy of exertional oxygen delivery by continuous versus demand-based flow systems during 6-minute walk test in patients with fibrotic interstitial lung disease and COPD in a hospital setting (OXYCODE): a protocol for a randomised trial.
Authors: Kronborg-White S, Jensen JH, Bendstrup E, Prior TS. BMJ Open 2025;15:e099664. doi:10.1136/bmjopen-2025-099664
Summary for clinicians: ERS guidelines on pulmonary alveolar proteinosis.
Authors: Alfaro T, McCarthy C, Bonella F, Bendstrup E, O’Callaghan M. Breathe 2025; 21(2):240224.
Complete Blood Cell Count Parameters Predict Mortality in Patients with Hypersensitivity Pneumonitis.
Authors: Katsaras M, Sotiropoulou V, Manali E, Fouka E, Bendstrup E, Kolilekas L, Tomos I, Papakosta D, Tzilas V, Steiropoulos P, Papanikolaou I, Ntolios P, Gogali A, Kostikas K, Tsiri P, Papaioannou O, Malakounidou E, Theohari E, Christopoulos I, Sampsonas F, Papiris SA, Rovina N, Bouros D, Tzouvelekis A. Diagnostics 2025, 15, 1038.
Diagnosis, screening, and follow-up of patients with familial interstitial lung disease: Results from an international survey.
Authors: Moen EV, Prior TS, Kreuter M, Wuyts WA, Molina-Molina M, Wijsenbeek M, Morais A, Tzouvelekis A, Ryerson CJ, Caro F, Buendia-Roldan I, Magnusson JM, Lee JS, Morisett J, Oldham JM, Troy LK, Funke-Chambour M, Alberti ML, Borie R, Walsh SLF, Rajan S, Kondoh Y, Khor YH, Bendstrup E. BMC Pulm Med. 2025 Feb 3;25(1):59.
Deciphering the pathogenicity of three NKX2-1 variants in ultra-severe forms of childhood interstitial lung disease.
Authors: Soreze, YD ; Desroziers-Louedec, T ; Carré, A ; Diab, F ; Daskalopoulou, A ; Starck, J & al , , PLoS One, 2025, 20, e0338446
Persistent tachypnoea of infancy and neuroendocrine cell hyperplasia of infancy: from systematic review to future directions
Authors: Nathan, N ; Grochowska, M ; Krenke, K ; Epaud, R ; Reix, P ; Griese, M & al , ., ERJ Open Res, 2025, 11,
Insights from a novel monogenic autoinflammatory disease: overview of a multicentric European cohort of 38 patients with COPA syndrome.
Authors: David, C ; Nathan, N ; Al-Abadi, E ;Arkwright, PD ; Bader-Meunier, B ; Becker, S & al Ann Rheum Dis, 2025, ,
French consensus statement on transition of adolescent and young adults with rare pulmonary disease from paediatric to adult care: a Delphi method study.
Authors: Peulier-Maitre, E ; Goutaki, M ; Nathan, N ; Patout, M ; Amedro, P ; Beltramo, G & al , , ERJ Open Res, 2025, 11
When Parents Refuse Sweat Test Despite a Positive Neonatal Blood Spot Screening for Cystic Fibrosis.
Authors: Nour, A ; Rusch, E ; Brégeaut, P ; Dubus, JC , , Acta Paediatr, 2025, ,
Reversal of Echocardiographic Alterations Following Hematopoietic Stem Cell Transplantation in Children With Sickle-Cell Anemia.
Authors: Riez, C ; Leroux, A ; Lopes, ME ; Kamdem, A ; Arnaud, C ; Hau, I & al , , Am J Hematol, 2025
Racial, Ethnic, Sex, and Gender Equity for Inclusive Interstitial Lung Disease Research: An Official American Thoracic Society Research Statement
Authors: Assayag, D ; Adegunsoye, A ; Khor, YH ; Bonella, F ; Borie, R ; Buendia, I & al Am J Respir Crit Care Med, 2025, 211, 2251-2267
Beyond Trikafta: new models to assess tissue dependent rescue of N1303K-CFTR Authors: Pranke, I ; Capurro, V ; Chevalier, B ; Pesce, E ; Tomati, V ; Pastorino, C & al . Front Pharmacol, 2025, 16, 1661417
Effectiveness of nirsevimab against hospitalisation for RSV-bronchiolitis during high RSV-B circulation in the second year of nationwide implementation in France: a test-negative case-control study.
Authors: Furgier, A ; Brehin, C ; Levy, C ; Basmaci, R ; Launay, E ; Jung, C & al , Lancet Reg Health Eur, 2025, 58, 101443
When simulation becomes physical: vicarious symptoms in standardized patients during osces
Authors: Rybak, A ; Decavele, M ; Taytard, J ; Allaire, M ; Sabourdin, N ; Serresse, L & al ., BMC Med Educ, 2025, 25, 1509
Phenotype-Genotype Correlations in ABCA3 Patients-The RespiRare Cohort.
Authors: Fleury, M ; Delestrain, C ; Hadchouel, A ; Mazenq, J ; Benhamida, M ; Bernard, AS & al , Pediatr Pulmonol, 2025, 60, e71324
Molecular Investigation in Early-Onset Interstitial Lung Disease: Results From 699 Unrelated Patients.
Authors: Louvrier, C ; Nathan, N ; Cottin, V, Desroziers, T ; Nau, V ; Soreze, Y & al , Respirology, 2025
The epigenomic landscape of bronchial epithelial cells reveals the establishment of trained immunity.
Authors: Bigot, J ; Legendre, R ; Hamroune, J ; Jacques, S ; Le Gars, M ; Millet, N & al , , Genes Immun, 2025, 26, 577-588
De novo SRRM2 variants in neuroendocrine cell hyperplasia of infancy and persistent tachypnea of infancy.
Authors: Louvrier, C ; Soreze, Y ; Mesinele, J ; de Becdelièvre, A ; Desroziers, T ; Nau, V & al , , Eur Respir J, 2025
Restoring chloride efflux in cystic fibrosis with TMEM16a antisense oligonucleotides.
Authors: Mitri, C ; Rousselet, N ; Bardin, P ; Dias Wickramanayaka, M ; Foussignière, T ; Dupuis, G & al , Mol Ther, 2025, 33, 6463-6478
Sweat chloride and lung function responses to elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis with two versus one responsive CFTR variants: an analysis of two real-world observational studies.
Authors: Burgel, PR ; Da Silva, J ; Girodon, E ; Durieu, I ; Reynaud-Gaubert, M ; Murris-Espin, M & al , Lancet Respir Med, 2025, 13, 978-989
[Guidelines from the SP2A (Pediatric Society of Pulmonology and Allergology) for the management of asthma in children aged 6 to 12years (without management of asthma exacerbation) – Short version]
Authors: Lejeune, S ; Wanin, S ; Amat, F ; Lezmi, G ; Deschildre, A ; Abou Taam, R & al Rev Mal Respir, 2025, 42, 418-433
Surfactant Protein (SP)-A Benefits Over SP-A Mutant: A Preliminary Study for Interstitial Lung Disease Treatment.
Authors: Desroziers, T ; Soreze, Y ; Legendre, M ; Dastot Le Moal, F ; Nau, V ; Amselem, S & al Am J Respir Cell Mol Biol, 2025, 73, 897-905
Natural variability of lung function in primary ciliary dyskinesia: longitudinal analysis from the PROVALF-PCD cohort.
Authors: Zhang, K ; Kant, A ; Boon, M ; Borrelli, M ; Constant, C ; Castillo Corullon, S & al, ERJ Open Res, 2025, 11,
International consensus statement on routine blood testing in primary ciliary dyskinesia
Authors: Altaraihi, S ; Marthin, JK ; Anagnostopoulou, P ; Boon, M ; Carr, SB ; Castillo-Corullón, S & al ERJ Open Res, 2025, 11
Younger Age and Viral Triggers Were Associated With Post-Infectious Bronchiolitis Obliterans in French Children.
Authors: Mazenq, J ; Crebassa, M ; Hadchouel, A ; Ghattas, S ; Reix, P ; Coutier, L & al Acta Paediatr, 2025, 114, 2900-2908
Penetrance of interstitial lung disease and lung cancer in carriers of SFTPA1 or SFTPA2 pathogenic variants.
Authors: Ducrot, L ; Nathan, N ; Benusiglio, PR ; Borie, R ; Nuel, G ; Legendre, M , . ERJ Open Res, 2025, 11,
Secretion management devices reduce hospitalizations for respiratory exacerbations in children with neurodisability.
Authors: Pradon, J ; Barzic, A ; Lubrano Lavadera, M ; Khirani, S ; Canavesio, C ; Fleurence, E & al. , Respir Med, 2025, 245, 108183
Growth-friendly Technique or Posterior Spinal Fusion With T-construct Pelvic Fixation in Nonambulatory Spinal Muscular Atrophy With Severe Scoliosis
Authors: Munro, APS ; Drysdale, SB ; Cathie, K ; Flamein, F ; Knuf, M ; Collins, AM & al. Lancet Child Adolesc Health, 2025, 9, 404-412
French protocol for diagnosis and management of type 1 interferonopathies.
Authors: Belot, A ; Boespflug-Tanguy, O ; Boursier, G ; Hully, M ; Neven, B ; Renaldo, F & al. F, Rev Med Interne, 2025, 46, 320-340
An update on diagnosis and treatments of childhood interstitial lung diseases.
Authors: Marczak, H ; Krenke, K ; Griese, M ; Carlens, J ; Seidl, E ; Gilbert, C & al. Breathe (Sheff), 2025, 21, 250004
Prenatal Diagnosis of Horseshoe Lung: A Report of Three Cases and Review of the Literature.
Authors: Birene, B ; Maurice, P ; Garel, C ; Prevost, B ; Soreze, Y ; Chabaud, M & al , , Prenat Diagn, 2025, 45, 1027-1038
Elexacaftor/Tezacaftor/Ivacaftor Population Pharmacokinetics in Pediatric Patients With Cystic Fibrosis.
Authors: Truong, NH ; Benaboud, S ; Bouazza, N ; Barboura, M ; Bardin, E ; Miralles, M & al. , Clin Transl Sci, 2025, 18, e70245
A non-resolving cough in a 41-year-old woman: a case of familial pulmonary fibrosis.
Authors: Carolan, A ; Ozaki, M ; Ng, WL ; Ryan, J ; O’Reilly, MW ; Dunne, R & al , Breathe (Sheff), 2025, 21, 240258
Allergic bronchopulmonary aspergillosis in cystic fibrosis: Case-control study from the French registry.
Authors: Tarizzo, M ; Lemonnier, L ; Leblanc, S ; Bigot, J ; Thouvenin, G ; Guillot, L & al. Med Mycol, 2025, 63,
Impact of A Multidisciplinary Team Discussion for Genetic Lung Fibrosis.
Authors: Franco, G ; Ba, I ; Nathan, N ; Guerin, C ; Lassus, A ; Kannengiesser, C & al. Respirology, 2025, 30, 523-532
Divergent responses to SARS-CoV-2 infection in bronchial epithelium with pre-existing respiratory diseases.
Authors: Oliva, J ; Ruffin, M ; Calmel, C ; Gibeaud, A ; Pizzorno, A ; Gaudin, C & al. , iScience, 2025, 28, 111999
PFMG2025-integrating genomic medicine into the national healthcare system in France. Lancet Reg Health Eur, 2025, 50, 101183
Diagnostic Evaluation and Clinical Findings in Children With Persistent Tachypnea of Infancy/Neuroendocrine Cell Hyperplasia of Infancy: A European Multicenter Retrospective
Authors: Marczak, H ; Krenke, K ; Griese, M ; Peradzy?ska, J ; Lange, J ; Kulus, M & al , Study., Chest, 2025, 168, 171-182
Efficacy and safety of CFTR modulators in patients with interstitial lung disease caused by ABCA3 transporter deficiency.
Authors: Le Brun, M ; Nathan, N ; Louvrier, C ; Legendre, M ; Feuillet, S ; Frija-Masson, J & al. , ERJ Open Res, 2025, 11,
Bi-allelic LAMP3 variants in childhood interstitial lung disease: a surfactant-related disease.
Authors: Louvrier, C ; Desroziers, T ; Soreze, Y ; Delgado Rodriguez, M ; Thomas, L ; Nau, V & al , , EBioMedicine, 2025, 113, 105626
Effect of Elexacaftor/Tezacaftor/Ivacaftor on Glucose Tolerance in Adolescents With Cystic Fibrosis.
Authors: Galderisi, A ; Weiss, L ; Besançon, A ; Stremler, N ; Reix, P ; Wizla, N & al. , J Clin Endocrinol Metab, 2025, 110, 3220-3229
Home noninvasive ventilation in pediatric patients: Does one size fit all?
Authors: Khirani, S ; Griffon, L ; Amaddeo, A ; Baudin, F ; Bierme, P ; Taytard, J & al , , Respir Med, 2025, 238, 107983
Healthcare worker views on antimicrobial resistance in chronic respiratory disease.
Authors: Ananth, S ; Adeoti, AO ; Ray, A ; Middleton, PG ; Ekkelenkamp, M ; Thee, S & al , , Antimicrob Resist Infect Control, 2025, 14, 1
Neuroendocrine tumor discovered in a teenager following asthma misdiagnosis.
Authors: Denamur, S ; Hervieux, E ; Peulier-Maitre, E ; Chalard, F ; Coulomb, A ; Nathan, N & al. , Arch Pediatr, 2025, 32, 72-76
Long term noninvasive respiratory support in children with OSA-I and OSA-II: Data of a nation-wide study.
Authors: Tommesani, C ; Khirani, S ; Amaddeo, A ; Massenavette, B ; Bierme, P ; Taytard, J & al. Sleep Med, 2025, 126, 67-72
Childhood interstitial lung disease survivors in adulthood: a European collaborative study
Authors: Manali, ED ; Griese, M ; Nathan, N ; Uzunhan, Y ; Borie, R ; Michel, K & al , ., Eur Respir J, 2025, 65,
Surfactant protein B deficiency: the RespiRare cohort.
Authors: Fleury, M ; Delestrain, C ; Roditis, L ; Perisson, C ; Renoux, MC ; Thumerelle, C & al , Thorax, 2025, 80, 109-112
Reversibility in plethysmographic airway and specific airway resistance in children.
Authors: Beydon, N et al. ERJ Open Res, 2025
Nerandomilast in Patients with Idiopathic Pulmonary Fibrosis.
Authors: Luca Richeldi. New England Journal of Medicine. 02/2025. doi: 10.1056/NEJMoa2414108.
FIBRONEER-ILD Trial Investigators. Nerandomilast in Patients with Progressive Pulmonary Fibrosis.
New Engl J Med. 05/2025. doi: 10.1056/NEJMoa2503643.
IMPALA-2 Trial Investigators. Phase 3 Trial of Inhaled Molgramostim in Autoimmune Pulmonary Alveolar Proteinosis.
Authors: Trapnell BC. N Engl J Med. 08/2025. doi: 10.1056/NEJMoa2410542.
Use of Telomere Length as a Biomarker in Idiopathic Pulmonary Fibrosis.
Authors: Dahlqvist C. Lung. 07/2025. doi: 10.1007/s00408-025-00830-6. PMID: 40632247
Lung involvement in rheumatoid arthritis, a prospective study.
Authors: Froidure A. ERJ Open Res. 09/2025. doi: 10.1183/23120541.01312-2024. PMID: 40959170
Childhood interstitial lung disease survivors in adulthood: a European collaborative study.
Authors: Manali ED. Eur Respir J. 02/2025. doi: 10.1183/13993003.00680-2024. PMID: 39638417
Immunoglobulin A mucosal immunity in people with cystic fibrosis shortly after initiation of highly effective modulator therapy.
Authors: Mottais A. ERJ Open Research. 11/2025. doi: 10.1183/23120541.01369-2024. PMID: 41220816
Total lung capacity is predictive of disease severity and survival in systemic sclerosis: A longitudinal analysis in 2347 patients from the French National Cohort Study.
Authors: Chaigne B., Bense A., Aubourg F., Agard C., Allanore Y., Berezne A., Pugnet G., Hachulla E., Cottin V., Hot A., Dunogue B., Kanagaratnam A., Palat S., Lescoat A., Berthier S., Chatelus E., Rivière S., Launay D., Truchetet M-E., Dinh-Xuan AT., Mouthon L. J Autoimmun. 03/2025. doi: 10.1016/j.jaut.2025.103391.
The moving target of progressive pulmonary fibrosis: insights for future clinical trials.
Authors: Cottin V. Eur Respir J. 03/2025. doi: 10.1183/13993003.00118-2025.
Treatable traits in interstitial lung disease: opportunities and challenges.
Authors: Khor YH, Podolanczuk AJ, Renzoni EA, McDonald VM, Cottin V, Holland AE, Inoue Y, Russell AM, Song JW, Wijsenbeek M, Strek ME, Ryerson CJ. Respir Med. 11/2025. doi: 10.1016/j.rmed.2025.108353.
Novel treatment strategies for lymphangioleiomyomatosis: a narrative review.
Authors: Elia D. Eur Respir Rev. 08/2025. doi: 10.1183/16000617.0019-2025.
Automated AI-based image analysis for quantification and prediction of interstitial lung disease in systemic sclerosis patients.
Authors: Guiot J. Respir Res. 01/2025. doi: 10.1186/s12931-025-03117-9.
Modulation of infiltrating CD206+ macrophages restricts progression of pulmonary lymphangioleiomyomatosis.
Authors: Liu HJ. Eur Respir J. 11/2025. doi: 10.1183/13993003.00084-2025.
Classification of interstitial pneumonias: ready for the future or stuck in the past?
Authors: Kolb M. Eur Respir J. 12/2025. doi: 10.1183/13993003.01845-2025.
Interstitial Lung Disease Associated with Anti-Ku Antibodies: A Case Series of 19 Patients.
Authors: Petitgrand L. J Clin Med. 01/2025. doi: 10.3390/jcm14010247.
Risk of hospitalisation and mortality among patients with interstitial lung disease and COVID-19: a French multicentre prospective cohort.
Authors: Diesler R. Pulmonology. 12/2025. doi: 10.1080/25310429.2025.2598693.
High-Throughput Proteomics in Lymphangioleiomyomatosis: Premelanosome Protein as a Diagnostic Biomarker, Construction of a Diagnosis Score, and Evidence of Neutrophil Involvement.
Authors: Young LR. Am J Respir Crit Care Med. 10/2025. doi: 10.1164/rccm.202506-1159OC.
Nintedanib Combined With Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis or Progressive Pulmonary Fibrosis: A Long-Term Retrospective Multicentre Study (Combi-PF).
Authors: Meersseman C. Arch Bronconeumol. 10/2025. doi: 10.1016/j.arbres.2025.10.004.1
Electrocardiogram-based detection of an atrial cardiomyopathy predicts postoperative supraventricular arrhythmias after lung transplantation.
Authors: Kuss. JTCVS Open. 09/2025. doi: doi: 10.1016/j.xjon.2025.
Additional diagnostic potential of transbronchial lung cryobiopsy in bronchoscopic assessment of sarcoidosis and chronic beryllium disease: a retrospective analysis of 321 patients.
Authors: Terschluse . BMJ Open Respir Res. 12/2025. doi: doi: 10.1136/bmjresp-2025-003206. NO
PDE-4 Inhibition in Sarcoidosis Patients: A Retrospective Single-Center Analysis of 51 Patients.
Authors: Feineis. Pharmaceuticals (Basel). 11/2025. doi: doi: 10.3390/ph18111729.
Coronary vasospasms and other cardiac manifestations in Eosinophilic Granulomatosis with Polyangiitis: Clinical impact and frequency in a monocentre study of 103 patients.
Authors: Schramm. RMD Open. 11/2025. doi: doi: 10.1136/rmdopen-2025-005816.
Evaluation of large language model-generated medical information on idiopathic pulmonary fibrosis.
Authors: Cherrez-Ojeda. Front Artif Intell. 09/2025. doi: 10.3389/frai.2025.1618378. eCollection 2025.
SARS-CoV-2 infection dynamics in a MHCI-mismatched lung transplant recipient.
Authors: Fuchs. Nat Commun. 09/2025. doi: doi: 10.1038/s41467-025-63681-y.
Progressive pulmonary fibrosis: current perspectives in diagnostic imaging.
Authors: Agarwal. BJR Open. 07/2025. doi: doi: 10.1093/bjro/tzaf018.
Beryllium Lymphocyte Proliferation Test: Differential Diagnosis of Sarcoidosis and Chronic Beryllium Disease.
Authors: Jouanjan. Chest. 12/2025. doi: doi: 10.1016/j.chest.2025.06.034.
Diagnosis and treatment of ANCA-associated vasculitis : S3 guideline of the German Society for Rheumatology and Clinical Immunology e. V. (DGRh) and German Society for Internal Medicine e. V. (DGIM), German Society for Nephrology e. V. (DGfN), German Society for ENT Medicine and Head and Neck Surgery e. V. (DGHNO-KHC), German Ophthalmological Society e. V. (DOG), German Society for Neurology e. V. (DGN), German Society for Pneumology and Respiratory Medicine e. V. (DGP), German Society for Pathology e. V. (DGP), German Radiological Society, Society for Medical Radiology e. V. (DRG), Federal Association of German Pathologists, Federal Kidney Association e. V., German Rheumatism League Federal Association e. V.
Authors: Holle. Z Rheumato. 04/2025. doi: 10.1007/s00393-024-01597-6.
The NEuroCOUGH Chronic Cough Registry: a protocol for a pan-European observational study.
Authors: S. P. Cho. ERJ Open Res. 09/2025. doi: doi: 10.1183/23120541.00289-2025.
Two heads are better than one: a synergistic strategy for conquering graft- versus-host disease.
Authors: Stolz. Haematologica. 07/2025. doi: doi: 10.3324/haematol.2025.287776.
AI-powered evaluation of lung function for diagnosis of interstitial lung disease.
Authors: Gompelmann. Thorax.. 06/2025. doi: doi: 10.1136/thorax-2024-221537.
Quantitative analysis of chest computed tomography in alpha-1-antitrypsin deficiency.
Authors: Höger. Respir Res. 11/2025. doi: doi: 10.1186/s12931-025-03413-4.
Aspergillus Serologic Findings and Clinical Outcomes in Patients With Bronchiectasis: Data From the European Bronchiectasis Registry.
Authors: Pollock. Chest. 04/2025. doi: 10.1016/j.chest.2024.06.3843. PMID: 39461553
Assessment of mTOR pathways in diffuse idiopathic neuroendocrine cell hyperplasia. Authors: O’Brien H, O’Callaghan M, Comerford L, McCormack J, Jouida A, Boyle N, O’Malley D, Worrell J, Franciosi AN, Byrne AJ, Fabre A, Keane MP, McCarthy C. Eur Respir J. 67/2026. doi: 10.1183/13993003.01692-2025
Pathogenic Glomulin Gene Variant in a Patient with Idiopathic Pulmonary Arterial Hypertension: A Novel Association Case Report.
Authors: Dimeas IE, Dimeas GE, Zakynthinos GE, McCarthy C, Daniil Z, Xiromerisiou G. Reports (MDPI). 8/2025. doi: 10.3390/reports8040209.
Fine-scale regional frequency of the MUC5B promoter variant correlates with healthcare burden of idiopathic pulmonary fibrosis.
Authors: Gilbert E, Carolan A, Ozaki M, Logan N, Musameh K, O’Brien H, Ng WL, Jasmin J, McCarthy C, Keane MP, Schwartz DA, Henry MT, Hurley K. ERJ Open Res. 11/2025. doi: 10.1183/23120541.00305-2025.
Phase 3 Trial of Inhaled Molgramostim in Autoimmune Pulmonary Alveolar Proteinosis.
Authors: Trapnell BC, Inoue Y, Bonella F, Wang T, McCarthy C, Arai T, Akasaka K, Mariani F, Mogulkoc N, Song JW, Baba T, Jouneau S, Numakura T, Öcal N, Mihaltan F, Ataya A, Bendstrup E, Campo I, Carey B, Arena R, … IMPALA-2 Trial Investigators. N Engl J Med. 393/2025. doi: 10.1056/NEJMoa2410542.
Small extracellular vesicles and induction of epithelial-to-mesenchymal transition in lymphangioleiomyomatosis.
Authors: Jouida A, Dantas LS, O’Malley D, Lynn E, O’Callaghan M, Guo M, McFadden P, Buckley G, Keane MP, McCarthy C. Eur Respir J. 66/2025. doi: 10.1183/13993003.02455-2024.
Summary for clinicians: ERS guidelines on pulmonary alveolar proteinosis.
Authors: Alfaro T, McCarthy C, Bonella F, Bendstrup E, O’Callaghan M. Breathe. 21/2025. doi: 10.1183/20734735.0224-2024.
Predicting Outcomes using DIGital TechnologY in patients with Interstitial Lung Disease (PRODIGY-ILD): Protocol for a Prospective Cohort Study.
Authors: Gunne E, Holden S, Franciosi AN, Keane M, McCarthy C, Doran P. BMJ Open. 15/2025. doi: 10.1136/bmjopen-2024-088271.
Deciphering the Role of Fluorodeoxyglucose-Positron Emission Tomography/CT Imaging in the Management of Sarcoidosis.
Authors: Donnelly R, Franciosi AN, Forde SH, McDermott M, Keane MP, Murphy DJ, McGrath EE, McCarthy C. Chest. 168/2025. doi: 10.1016/j.chest.2025.02.017.
The healthcare burden of pulmonary alveolar proteinosis (PAP).
Authors: Lee E, Ataya A, McCarthy C, Godart E, Cosenza J, King A, Robinson B, Wang T. Orphanet J Rare Dis. 20/2025. doi: 10.1186/s13023-024-03478-2.
Does Adenosine Triphosphate via Purinergic Receptor Signalling Fuel Pulmonary Fibrosis?
Authors: Forde L, Gogoi D, Baird R, McCarthy C, Keane MP, Reeves EP, McGrath EE. J Innate Immun. 17/2025. doi: 10.1159/000543083.
Childhood interstitial lung disease survivors in adulthood: a European collaborative study.
Authors: Manali ED, Griese M, Nathan N, Uzunhan Y, Borie R, Michel K, Schwerk N, Fijolek J, Radzikowska E, Chua F, Pabary R, Mogulkoc N, McCarthy C, Kallieri M, Papaioannou AI, Kiper N, Koziar Vasakova M, Lacina L, Molina-Molina M, Torrent-Vernetta A, … Papiris SA. Eur Respir J. 65/2025. doi: 10.1183/13993003.00680-2024.O’Malley, D., Byrne, A. J., Keane, M. P., & McCarthy, C. (2025). Lymphangioleiomyomatosis and its Treatment: When to Start?. Archivos de bronconeumologia, 61(1), 1–2. https://doi.org/10.1016/j.arbres.2024.09.005
Reply: The rationale for distinguishing RB-ILD and AMP.
Authors: Piciucchi S, Adegunsoye A, Travis WD, Nicholson AG, Wells AU, Cooper WA, Khor YH, Wijsenbeek MS, Sharma A, Hariri LP, Antoniou K, Borie R, Fabre A, Inoue Y, Johannson KA, Johkoh T, Kawano-Dourado L, Kazerooni E, Maher TM, Molyneaux PL, … Ryerson CJ. Eur Respir J. 66/2025. doi: 10.1183/13993003.02029-2025.
ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease developed by the task force for connective tissue disease-associated interstitial lung disease of the European Respiratory Society (ERS) and the European Alliance of Associations for Rheumatology (EULAR) Endorsed by the European Reference Network on rare respiratory diseases (ERN-LUNG).
Authors: Antoniou KM, Distler O et al., Ann Rheum Dis. 85/2026. doi: 10.1016/j.ard.2025.08.021.
Update of the international multidisciplinary classification of the interstitial pneumonias: an ERS/ATS statement.
Authors: Ryerson CJ et al., Eur Respir J. 66/2025. doi: 10.1183/13993003.00158-2025
Adverse side-effects of antifibrotic drugs and outcome of treatment in idiopathic pulmonary fibrosis.
Authors: Jovanovic D, Mogulkoc N, Sterclova M, Vasakova MK. J Thorac Dis.. 12/2025. doi: 10.21037/jtd-2025-130.
Lung Transplantation in Idiopathic Pulmonary Fibrosis Patients in the European MultiPartner IPF Registry: Challenges for Health Equity.
Authors: Tóth NM, Kramer MR, Šterclová M, Vašáková MK. Biomedicines. 10/2025. doi: 10.3390/biomedicines13112684.
Clinical Characteristics and Survival of Patients with Idiopathic Pulmonary Fibrosis: Analysis of the Serbian Cohort from the EMPIRE Registry.
Authors: Dimic-Janjic S, Stjepanovic M, Belic S, Vasakova MK. Diagnostics (Basel). 2025. 08/2025. doi: 10.3390/diagnostics15172121.
The necessity of geneticist and pulmonologist collaboration in the treatment of monogenic interstitial lung diseases in adults.
Authors: Sterclova M, Doubkova M, Doubek M. Breathe (Sheff). 08/2025. doi: 10.1183/20734735.0255-2024.
Serum Specific Immunoglobulins G. Do They Belong to the Standard Diagnostic Workup in Interstitial Lung Diseases?
Authors: Koziar Vasakova M, Sterclova M. Arch Bronconeumol.. 04/2024. doi: 10.1016/j.arbres.2025.01.012.
Up-to-date therapy of interstitial lung processes.
Authors: Sterclova M. Vnitr Lek.. 02/2025. doi: 10.36290/vnl.2025.039.
Anti-MDA5 Dermatomyositis–Associated Interstitial Lung Disease: clinical phenotype and response to therapy.
Authors: Gono T. Eur Respir J. 02/2025. doi: 10.1183/13993003.00567-2024.
Design of an open-label extension trial of nerandomilast (BI 1015550) in patients with idiopathic pulmonary fibrosis and progressive pulmonary fibrosis (FIBRONEER™-ON).
Authors: Wuyts W.A.. BMC PULMONARY MEDICINE. 12/2025. doi: 10.1186/s12890-025-03973-7.
A deep generative model for deciphering cellular dynamics and in silico drug discovery in complex diseases.
Authors: Wuyts W.A.. NATURE BIOMEDICAL ENGINEERING. 06/2025. doi: 10.1038/s41551-025-01423-7.
Survey of physicians’ approaches to the clinical evaluation of interstitial lung disease in Singapore (SoPhoCLES).
Authors: Wuyts W.A.. JOURNAL OF THORACIC DISEASE. 11/2025. doi: 10.21037/jtd-2025-1365.
Newly Onset Dyspnea During Pregnancy.
Authors: Wuyts W.A.. CHEST. 10/2025. doi: 10.1016/j.chest.2025.05.040.
Effect of Admilparant, a Lysophosphatidic Acid Receptor 1 Antagonist, on Disease Progression in Pulmonary Fibrosis.
Authors: Wuyts W.A.. CHEST. 09/2025. doi: 10.1016/j.chest.2025.04.003.
Stratifying GAP Stages by MUC5B rs35705950 T-Allele Carriage Refines Survival Prediction in IPF.
Authors: Wuyts W.A.. LUNG. 07/2025. doi: 10.1007/s00408-025-00837-z.
ABCE1: A new cytoplasmic autoantibody target in systemic sclerosis-associated interstitial lung disease.
Authors: Wuyts W.A.. AUTOIMMUNITY REVIEWS. 04/2025. doi: 10.1016/j.autrev.2025.103764.
Epigenetic age acceleration in idiopathic pulmonary fibrosis revealed by DNA methylation clocks.
Authors: Wuyts W.A.. AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY. 03/2025. doi: 10.1152/ajplung.00171.2024.
Person-centred health outcomes in the routine care for people with progressive pulmonary fibrosis: the COCOS-IPF project’s European survey on healthcare professionals’ views and practices.
Authors: Wuyts W.A.. RESPIRATORY RESEARCH. 02/2025. doi: 10.1186/s12931-025-03146-4.
Diagnosis, screening, and follow-up of patients with familial interstitial lung disease: Results from an international survey.
Authors: Wuyts W.A.. BMC PULMONARY MEDICINE. 02/2025. doi: 10.1186/s12890-025-03532-0.
Continued Treatment with Nintedanib in Patients with Progressive Pulmonary Fibrosis: Data from INBUILD-ON.
Authors: Wuyts W.A.. LUNG. 01/2025. doi: 10.1007/s00408-024-00778-z.
Identifying outcome domains to establish a core outcome set for progressive pulmonary fibrosis: a scoping review.
Authors: Wuyts W.A.. EUROPEAN RESPIRATORY REVIEW. 01/2025. doi: 10.1183/16000617.0133-2024.
2024
Interstitial Lung Disease Associated with Systemic Sclerosis.
Authors: Mismetti V, Si-Mohamed S, Cottin V.Semin Respir Crit Care Med. 2024 Jun;45(3):342-364. doi: 10.1055/s-0044-1786698. Epub 2024 May 7.PMID: 38714203 Review.
Evidence from recent clinical trials in fibrotic interstitial lung diseases.
Authors: Cottin V, Valenzuela C.Curr Opin Pulm Med. 2024 Sep 1;30(5):484-493. doi: 10.1097/MCP.0000000000001089. Epub 2024 Jul 4.PMID: 39114938 Review.
The Role of Inflammation and Fibrosis in Interstitial Lung Disease Treatment Decisions.
Authors: Behr J, Salisbury ML, Walsh SLF, Podolanczuk AJ, Hariri LP, Hunninghake GM, Kolb M, Ryerson CJ, Cottin V, Beasley MB, Corte T, Glanville AR, Adegunsoye A, Hogaboam C, Wuyts WA, Noth I, Oldham JM, Richeldi L, Raghu G, Wells AU.Am J Respir Crit Care Med. 2024 Aug 15;210(4):392-400. doi: 10.1164/rccm.202401-0048PP.PMID: 38484133 No abstract available.
Combined pulmonary fibrosis and emphysema syndrome: the age of majority.
Authors: Cottin V.Eur Respir J. 2024 Apr 4;63(4):2400353. doi: 10.1183/13993003.00353-2024. Print 2024 Apr.PMID: 38575167 No abstract available.
Bexotegrast in Patients with Idiopathic Pulmonary Fibrosis: The INTEGRIS-IPF Clinical Trial.
Authors: Lancaster L, Cottin V, Ramaswamy M, Wuyts WA, Jenkins RG, Scholand MB, Kreuter M, Valenzuela C, Ryerson CJ, Goldin J, Kim GHJ, Jurek M, Decaris M, Clark A, Turner S, Barnes CN, Achneck HE, Cosgrove GP, Lefebvre ÉA, Flaherty KR; PLN-74809-IPF-202 Trial Investigators.Am J Respir Crit Care Med. 2024 Aug 15;210(4):424-434. doi: 10.1164/rccm.202403-0636OC.PMID: 38843105 Free PMC article. Clinical Trial.
C-reactive protein as a candidate biomarker in fibrotic interstitial lung disease.
Authors: Cottin V, Valenzuela C.Respirology. 2024 Mar;29(3):195-198. doi: 10.1111/resp.14666. Epub 2024 Jan 31.PMID: 38296837 Free article. No abstract available.
High risk of lung cancer in surfactant-related gene variant carriers.
Authors: Brudon A, Legendre M, Mageau A, Bermudez J, Bonniaud P, Bouvry D, Cadranel J, Cazes A, Crestani B, Dégot T, Delestrain C, Diesler R, Epaud R, Philippot Q, Théou-Anton N, Kannengiesser C, Ba I, Debray MP, Fanen P, Manali E, Papiris S, Nathan N, Amselem S, Gondouin A, Guillaumot A, Andréjak C, Jouneau S, Beltramo G, Uzunhan Y, Galodé F, Westeel V, Mehdaoui A, Hirschi S, Leroy S, Marchand-Adam S, Nunes H, Picard C, Prévot G, Reynaud-Gaubert M, De Vuyst P, Wemeau L, Defossez G, Zalcman G, Cottin V, Borie R; OrphaLung network.Eur Respir J. 2024 May 2;63(5):2301809. doi: 10.1183/13993003.01809-2023. Print 2024 May.PMID: 38575158 Free PMC article.
Genetics and Genomics of Pulmonary Fibrosis: Charting the Molecular Landscape and Shaping Precision Medicine.
Authors: Adegunsoye A, Kropski JA, Behr J, Blackwell TS, Corte TJ, Cottin V, Glanville AR, Glassberg MK, Griese M, Hunninghake GM, Johannson KA, Keane MP, Kim JS, Kolb M, Maher TM, Oldham JM, Podolanczuk AJ, Rosas IO, Martinez FJ, Noth I, Schwartz DA.Am J Respir Crit Care Med. 2024 Aug 15;210(4):401-423. doi: 10.1164/rccm.202401-0238SO.PMID: 38573068 Review.
Pulmonary fibrosis is uncommon in primary Sjögren disease.
Si-Mohamed SA, Cottin V.Diagn Interv Imaging. 2024 May;105(5):163-164. doi: 10.1016/j.diii.2024.03.002. Epub 2024 Mar 15.PMID: 38493045 No abstract available.
[Genetic diffuse cystic lung disease in adults].
Diesler R, Ahmad K, Chalabreysse L, Glérant JC, Harzallah I, Touraine R, Si-Mohamed S, Cottin V.Rev Mal Respir. 2024 Jan;41(1):69-88. doi: 10.1016/j.rmr.2023.08.005. Epub 2023 Nov 10.PMID: 37951745 Review. French.
Quantitative Imaging Methods in Combined Pulmonary Fibrosis and Emphysema.
Wang JM, Araki T, Cottin V, Han MK, Oldham JM.Chest. 2024 Dec;166(6):1463-1472. doi: 10.1016/j.chest.2024.08.007. Epub 2024 Aug 16.PMID: 39154797 Review.
Chronic interstitial lung disease associated with systemic lupus erythematosus: A multicentric study of 89 cases.
Deneuville L, Mageau A, Debray MP, Sacre K, Costedoat-Chalumeau N, Hachulla E, Uzunhan Y, Le Tallec E, Cadranel J, Marchand Adam S, Montani D, Rémi-Jardin M, Reynaud-Gaubert M, Prevot G, Beltramo G, Crestani B, Cottin V, Borie R; OrphaLung Network.Respirology. 2024 Aug;29(8):713-721. doi: 10.1111/resp.14703. Epub 2024 Mar 17.PMID: 38494831 Free article.
Impact of Air Pollution and MUC5B Genotype on Survival in Idiopathic Pulmonary Fibrosis.
Sesé L, Borie R, Kannengiesser C, Cottin V, Israel-Biet D, Crestani B, Cadranel J, Chenivesse C, Boubaya M, Valeyre D, Annesi-Maesano I, Nunes H; COFI collaborators.Ann Am Thorac Soc. 2024 Mar;21(3):519-523. doi: 10.1513/AnnalsATS.202305-495RL.PMID: 38096447 No abstract available.
Rituximab and mycophenolate mofetil in interstitial lung disease (EVER-ILD): 1-year follow-up results of a randomised controlled trial.
Mansy L, Caille A, Reynaud-Gaubert M, Bermudez J, Bonniaud P, Borie R, Brillet PY, Cadranel J, Court-Fortune I, Crestani B, Debray MP, Duprez M, Guillaumot A, Hirschi-Santelmo S, Israel-Biet D, Jouneau S, Juvin K, Kerjouan M, Mankikian J, Marquette CH, Naccache JM, Nunes H, Plantier L, Prevot G, Quetant S, Traclet J, Valentin V, Uzunhan Y, Wémeau-Stervinou L, Cottin V, Marchand-Adam S; EVER-ILD investigators and the OrphaLung network.Eur Respir J. 2024 Sep 12;64(3):2401368. doi: 10.1183/13993003.01368-2024. Print 2024 Sep.PMID: 39231630 Clinical Trial. No abstract available.
Mepolizumab and benralizumab in patients with severe asthma and a history of eosinophilic granulomatosis with polyangiitis.
Desaintjean C, Ahmad K, Traclet J, Gerfaud-Valentin M, Durel CA, Glerant JC, Hot A, Lestelle F, Mainbourg S, Nasser M, Seve P, Turquier S, Devouassoux G, Cottin V.Front Med (Lausanne). 2024 Mar 22;11:1341310. doi: 10.3389/fmed.2024.1341310. eCollection 2024.PMID: 38585151 Free PMC article.
Similarities and differences of interstitial lung disease associated with pathogenic variants in SFTPC and ABCA3 in adults.
Diesler R, Legendre M, Si-Mohamed S, Brillet PY, Wemeau L, Manali ED, Gagnadoux F, Hirschi S, Lorillon G, Reynaud-Gaubert M, Bironneau V, Blanchard E, Bourdin A, Dominique S, Justet A, Macey J, Marchand-Adam S, Morisse-Pradier H, Nunes H, Papiris SA, Traclet J, Traore I, Crestani B, Amselem S, Nathan N, Borie R, Cottin V; OrphaLung network.Respirology. 2024 Apr;29(4):312-323. doi: 10.1111/resp.14667. Epub 2024 Feb 12.PMID: 38345107 Free article.
The fibroblast activation protein alpha as a biomarker of pulmonary fibrosis.
Lavis P, Garabet A, Cardozo AK, Bondue B.Front Med (Lausanne). 2024 Sep 19;11:1393778. doi: 10.3389/fmed.2024.1393778. eCollection 2024.PMID: 39364020 Free PMC article. Review.
A formula for predicting emphysema extent in combined idiopathic pulmonary fibrosis and emphysema.
Wells AU, Jacob J, Sverzellati N, Cross G, Barnett J, De Lauretis A, Antoniou K, Weycker D, Atwood M, Kirchgaessler KU, Cottin V.Respir Res. 2024 Jan 18;25(1):33. doi: 10.1186/s12931-023-02589-x.PMID: 38238788 Free PMC article.
Clinical course of suspected familial and sporadic idiopathic pulmonary fibrosis: Data from the PROOF-Next registry.
Froidure A, Bondue B, Dahlqvist C, Guiot J, Gusbin N, Wirtz G, Brusselle G, Strens D, Slabbynck H, Wuyts WA.Respirology. 2024 Apr;29(4):304-311. doi: 10.1111/resp.14650. Epub 2023 Dec 20.PMID: 38123492 Free article.
Phenotypes and outcome of diffuse pulmonary non-amyloid light chain deposition disease.
Lestelle F, Beigelman C, Rotzinger D, Si-Mohamed S, Nasser M, Wemeau L, Hirschi S, Prevot G, Roux A, Bunel V, Gomez E, Sohier L, Pradier HM, Gaubert MR, Gondouin A, Lazor R, Glerant JC, Bejui FT, Colombat M, Cottin V; OrphaLung network.Respir Res. 2024 Apr 10;25(1):159. doi: 10.1186/s12931-024-02798-y.PMID: 38600600 Free PMC article.
The IgA-pIgR System Is Dysregulated in Idiopathic Pulmonary Fibrosis.
Planté-Bordeneuve T, Bertrand Y, Lecocq M, Hoton D, Fillée C, Lacroix V, Rondelet B, Wuyts W, Bouzin C, Pilette C, Froidure A.Am J Respir Crit Care Med. 2024 Sep 15;210(6):841-844. doi: 10.1164/rccm.202401-0043LE.PMID: 38976848 No abstract available.
Bexotegrast in people with idiopathic pulmonary fibrosis (IPF): a plain language summary of publication of the INTEGRIS-IPF study.
Lancaster L, Cottin V, Ramaswamy M, Wuyts WA, Jenkins RG, Scholand MB, Kreuter M, Valenzuela C, Ryerson CJ, Goldin J, Kim GHJ, Jurek M, Decaris M, Clark A, Turner SM, Barnes CN, Achneck HE, Cosgrove GP, Lefebvre ÉA, Flaherty KR.Ther Adv Respir Dis. 2024 Jan-Dec;18:17534666241287307. doi: 10.1177/17534666241287307.PMID: 39512136 Free PMC article. Review.
A 24/7 Pilot Remote Emergency Multidisciplinary Discussion for Rapidly Progressive Interstitial Lung Disease: A 2-Year Experience.
Bay P, Pineton de Chambrun M, Allenbach Y, Le Pavec J, Picard C, Zuber B, Bunel V, Hervier B, Meyer A, Miyara M, Brillet PY, Boussouar S, Declercq C, Tandjaoui-Lambiotte Y, Nunes H, Cottin V, Hachulla E, Uzunhan Y; OrphaLung, RespiFIL, and FAI2R Networks.Chest. 2024 Aug 27:S0012-3692(24)05053-0. doi: 10.1016/j.chest.2024.07.179. Online ahead of print.PMID: 39197513 No abstract available.
Quantitative CT-analysis of over aerated lung tissue and correlation with fibrosis extent in patients with idiopathic pulmonary fibrosis.
Tonelli R, Smit MR, Castaniere I, Casa GD, Andrisani D, Gozzi F, Bruzzi G, Cerri S, Samarelli AV, Raineri G, Spagnolo P, Rizzoni R, Ball L, Paulus F, Bos LDJ, Clini E, Marchioni A.Respir Res. 2024 Oct 5;25(1):359. doi: 10.1186/s12931-024-02970-4.PMID: 39369240 Free PMC article.
Pulmonary lymphangiomatosis: insights into an ultra-rare disease.
Polke M, Polke N, Piel S, Brunnemer E, Wälscher J, Buschulte K, Warth A, Heussel CP, Eichinger M, Frankenstein L, Eichhorn M, Miliauskas S, Herth FJF, Kreuter M.Respir Res. 2024 Nov 26;25(1):416. doi: 10.1186/s12931-024-03040-5.PMID: 39593123 Free PMC article.
Ultra-low dose chest CT for the diagnosis of pulmonary arteriovenous malformation in patients with hereditary hemorrhagic telangiectasia.
Delpon JE, Greffier J, Lacombe H, Barbe A, Bouin M, De Oliveira F, Mansuy A, Delagrange L, Fargeton AE, Beregi JP, Cottin V, Dupuis-Girod S, Si-Mohamed SA.Diagn Interv Imaging. 2024 Oct;105(10):364-370. doi: 10.1016/j.diii.2024.03.006. Epub 2024 Apr 10.PMID: 38604894 Free article.
Childhood interstitial lung disease survivors in adulthood: a European collaborative study.
Manali ED, Griese M, Nathan N, Uzunhan Y, Borie R, Michel K, Schwerk N, Fijolek J, Radzikowska E, Chua F, Pabary R, Mogulkoc N, McCarthy C, Kallieri M, Papaioannou AI, Kiper N, Koziar Vasakova M, Lacina L, Molina-Molina M, Torrent-Vernetta A, Tsiligiannis T, Karadag B, Kokosi M, Renzoni EA, Hm van Moorsel C, Campo I, Bendstrup E, Skovhus Prior T, Prasse A, Bonella F, Cottin V, Diesler R, Froidure A, Kolilekas L, Fotis L, Douros K, Kaditis AG, Jeny F, Chauveau S, Nunes H, Dahbia A, Mariani F, van der Vis JJ, Groen K, Erdem Eralp E, Gokdemir Y, Kocakaya D, Olgun Yildizeli S, Yalçın E, Emiralioğlu N, Nayir Buyuksahin H, O’Brien H, Karcıoglu O, Can D, Ezircan A, Kartal Ozturk G, Ocal N, Yuksel H, Narin Tongal S, Safrankova M, Kourtesi K, Louvrier C, Kannengiesser C, Fabre A, Legendre M, Crestani B, Pohunek P, Bush A, Papiris SA.Eur Respir J. 2024 Dec 5:2400680. doi: 10.1183/13993003.00680-2024. Online ahead of print.PMID: 39638417
The impact of time from ILD diagnosis to referral to the transplant center on the probability of inclusion in the transplant waiting list.
Mora-Cuesta VM, Martínez-Meñaca A, González-Fernández Á, Iturbe-Fernández D, Tello-Mena S, Izquierdo-Cuervo S, Fernández-Rozas S, Alonso-Lecue P, Cifrián-Martínez JM.Heart Lung. 2024 Sep-Oct;67:92-99. doi: 10.1016/j.hrtlng.2024.04.022. Epub 2024 May 11.PMID: 38735159
Pulmonary fibrosis in patients with autoimmune pulmonary alveolar proteinosis: a retrospective nationwide cohort study.
Guirriec Y, Luque-Paz D, Bernard G, Mabo A, Kerjouan M, Ménard C, Monnier D, Nunes H, Uzunhan Y, Reynaud-Gaubert M, Bermudez J, Borie R, Crestani B, Traclet J, Wémeau-Stervinou L, Chenivesse C, Gomez E, Prévot G, Bourdin A, Bondue B, Bergeron A, Cottin V, Lederlin M, Jouneau S; OrphaLung network.ERJ Open Res. 2024 Dec 2;10(6):00314-2024. doi: 10.1183/23120541.00314-2024. eCollection 2024 Nov.PMID: 39624377 Free PMC article.
Characteristics and outcomes of patients with LAM receiving sirolimus in France based on real-life data.
Cottin V, Cases A, Bourdin V, Reynaud-Gaubert M, Hirschi S, Kerjouan M, Diesler R, Chardès B, Fiévez S, Assi N, Schmidt A, Denis H, Wémeau-Stervinou L, Uzunhan Y.Front Med (Lausanne). 2025 Jan 8;11:1494713. doi: 10.3389/fmed.2024.1494713. eCollection 2024.PMID: 39845810 Free PMC article.
Evaluation of efficacy and safety of rituximab in patients with progressive interstitial lung disease (ILD) with inflammatory component (EvER-ILD2): A multicentre double-blind placebo-controlled randomized trial.
Authors: Ferreira M, Bejan-Angoulvant T, Marchand-Adam S, Mousset E, Mureau E, Jouneau S, Nunes H, Montani D, Chenivesse C, Cadranel J, Bonniaud P, Crestani B, Cottin V, Caille A; OrphaLung..Respir Med Res. 2024 Nov 28;87:101144. doi: 10.1016/j.resmer.2024.101144. Online ahead of print.PMID: 39693827
Authors: Matt MG, Drozdov D, Bendstrup E, Glerup M, Hauge EM, Masmas T, Schneider EC, Zeilhofer UB, Abu-Arja RF, Driest KD, Oved JH, Onel K, Ebens CL, Chellapandian D, Chandrakasan S, Prahalad S, Roth J, Prockop SE, Silva J, Schapiro AH, Towe C, Chandra S, Grom A, Schulert GS, Marsh RA. Lancet Rheumatol. 2024 Dec 20:S2665-9913(24)00275-3.
Authors: Petrarulo S, Ravaglia C, Kronborg-Whote S, Madsen LB, Lex F, Dubini A, Fabbri E, Bendstrup E, Spagnolo P, Piciucchi A, Poletti V. Pulmonology; 31: 2425503, 2025.
Authors: Luppi F, Manfredi A, Faverio P, Franco G, Salvarani C, Bendstrup E, Sebastiani M. Autoimmunity Review 2024; 23: 103668.
Authors: Lough G, Abdulqawi R, Amanda G, Antoniou K, Azuma A, Baldi M, Bayoumy A, Behr J, Bendstrup E, Bouros D, Brown K, Chaudhuri N, Corte TJ, Cottin V, Crestani B, Flaherty KR, Glaspole I, Kawano-Dourado L, Keane MP, Kolb M, Martinez FJ, Molina-Molina M, Ojanguren I, Pearmain L, Raghu G, Rottoli P, Stanel SC, Tabaj G, Vancheri C, Varela B, Wang B, Wells A, Rivera-Ortega P. ERJ Open Res. 2024.
Authors: Montaño C, Bendstrup E, Rønnov-Jessen I, et al. ERJ Open Res 2024; in press(https://doi.org/10.1183/23120541.00567-2024).
Authors: Matteson EL, Bendstrup E, Strek ME, Dieudé P. ACR Open Rheumatology Vol. 0, No. 0, Month 2024, pp 1–10. DOI 10.1002/acr2.11736
Authors: McCarthy C, Bonella FV, O’Callaghan M, Dupin C, Alfaro T, Fally M, Borie R, Campo I, Cottin , Fabre A, Griese M, Hadchouel A, Jouneau S, Kokosi M, Manali E, Prosch H, Trapnell B, Veltkamp M, Wang T, Toews I, Mathioudakis A, Bendstrup E. Eur Respir J 2024; in press (https://doi.org/10.1183/13993003.00725-2024).
Authors: Kalluri M, Moitra S, Richman-Eisenstat J, Ferrara G, Bendstrup E, Maarsaa K. Eur. Resp. J. 2024; 64: 2301956 [DOI: 10.1183/13993003.01956-2023].
Authors: Olesen SR, Kronborg-White S, Madsen LB, Bendstrup E. Eur Clin Respir J. 2024 Jun 18;11(1):2365510. doi: 10.1080
Authors: Iversen IB, Vestergaard JM, Ohlander J, Peters S, Bendstrup E, Bonde JPE, Schlünssen V, Bønløkke JH, Rasmussen F, Stokholm ZA, Andersen MB, Kromhout H, Kolstad HA.
Authors: Prior TS, Hoyer N, Davidsen JR, Shaker SB, Hundahl PM, Lomholt S, Deleuran BW, Bendstrup E, Kragstrup TW. Scand J Immunol. 2024;00:e13392.
Authors: Iversen IB, Vestergaard JM, Basinas I, Ohlander J, Peters S, Bendstrup E, Bonde JPE, Schlünssen V, Rasmussen F, Stokholm ZA, Andersen MB, Kromhout H, Kolstad HA. Thorax 2024; 2024;0:1–8. doi:10.1136/thorax-2023-221275.
Authors: Iversen IB, Vestergaard JM, Ohlander J, Peters S, Bendstrup E, Bonde JPE, Schlünssen V, Bønløkke JH, Rasmussen F, Stokholm ZA, Andersen MB, Kromhout H, Kolstad HA. . Scand J Work Environ Health 2024.
Authors: Bendstrup E, Lynn E, Troldborg A. Semin Respir Crit Care Med 2024.
Authors: Kirkeby MH, Bendstrup E, Rose HK. Front. Med. 11:1328471. doi: 10.3389/fmed.2024.1328471
Authors: Elkjær AL, Næser EU, Aaen KT, Hovgaard HL, Juhl Olsen P, Bendstrup E, Søndergaard K. Rheumatology International https://doi.org/10.1007/s00296-024-05532-5
Authors: Hvelplund CY, Refsgaard B, Bendstrup E. American Journal of Hospice & Palliative Medicine® 2024, Vol. 0(0) 1–7.
Authors: Kristensen K, Janne Møller J, Terkelsen AJ, Harbo T, Hansen IT, Gormsen LC, Bendstrup E. 2023; 40 (4): e2023052.
Authors: Lynn E, Forde SH, Franciosi AN, Bendstrup E, Veltkamp M, Wind AE, Van Moorsel CHM, Lund TK, Durheim MT, Peeters EFHI, Keane MP, McCarthy C. Am J Respir Crit Care Med. 2024 Feb 15;209(4):456-459.o
Authors: Boccard, V ; Corvol, F ; Prévost, B ; Corvol, H , , BMJ Case Rep, 2024, 17
Similarities and differences of interstitial lung disease associated with pathogenic variants in SFTPC and ABCA3 in adults
Authors: Diesler, R ; Legendre, M ; Si-Mohamed, S ; Brillet, PY ; Wemeau, L ; Manali, ED & al. Respirology, 2024, 29, 312-323
Authors: Denamur, S ; Hervieux, E ; Cosson, L ; Sileo, C ; Coulomb, A ; Nathan, N & al , Pediatr Pulmonol, 2024, 59, 1095-1098
Lung biopsies in infants and children in critical care situation
Authors: Levy, Y ; Bitton, L ; Sileo, C ; Rambaud, J ; Soreze, Y ; Louvrier, C & al., Pediatr Pulmonol, 2024, 59, 907-914
Pediatric long-term noninvasive respiratory support in children with central nervous system disorders
Authors: Carrara, M ; Aubertin, G ; Khirani, S ; Massenavette, B ; Bierme, P ; Griffon, L & al Pediatr Pulmonol, 2024, 59, 642-651
Dysplasia in a Full-Term Newborn with a NKX2.1 Variant
Authors: Soreze, Y ; Nathan, N ; Jegard, J ; Hervieux, E ; Clermidi, P ; Sileo, C & al , Acinar., Neonatology, 2024, 121, 133-136
The significance of multidisciplinary team meetings in diagnosing and managing childhood interstitial lung disease within the RespiRare network
Authors: Cassibba, J ; Epaud, R ; Berteloot, L ; Aberbache, S ; Bitton, L ; Fletcher, C & al. Pediatr Pulmonol, 2024, 59, 417-425
Lung transplantation as a rescue option in childhood critical pulmonary Langerhans cell histiocytosis
Authors: Heritier, S ; Donadieu, J ; Leger, PL ; De Tersant, M ; Della Vallee, V ; Barkaoui, MA & al , Pediatr Pulmonol, 2024, 59, 192-195
Unusual cause of dyspnea in patient with Myelofibrosis: The Ruxolitinib lung. Respiratory Medicine Case Reports 2024
Authors: El Kik A,Vander Kuylen M, Bailly B, Fallas J, Bondue B.; 51: 102090.
Usefulness of FAPα assessment in bronchoalveolar lavage as a marker of fibrogenesis: results of a preclinical study and first report in patients with idiopathic pulmonary fibrosis.
Authors: Lavis P, Pingitore J, Doumont G, Garabet A, Van Simaeys G, Lacroix S, Passon N, Van Heymbeek C, De Maeseneire C, Allard J, Collin A, Huaux F, Decaestecker C, Salmon I, Goldman S, Cardozo AK, Bondue B. Respir Res. 2023 Oct 25;24(1):254. doi: 10.1186/s12931-023-02556-6.
ERS statement on transition of care in childhood interstitial lung diseases
Authors: Pohunek P, Manali E, Vijverberg S, Carlens J, Chua F, Epaud R, Gilbert C, Griese M, Karadag B, Kerem E, Koucký V, Nathan N, Papiris S, Terheggen-Lagro S, Plch L, Torrent Vernetta A, Bush A.. Eur Respir J. 2024 Aug 15;64(2):2302160. doi: 10.1183/13993003.02160-2023. PMID: 38843911 : ERS statement on transition of care in childhood interstitial lung diseases – PubMed
Recommendations for asthma monitoring in children: A PeARL document endorsed by APAPARI, EAACI, INTERASMA, REG, and WAO
Authors: Papadopoulos NG, Custovic A, Deschildre A, Gern JE, Nieto Garcia A, Miligkos M, Phipatanakul W, Wong G, Xepapadaki P, Agache I, Arasi S, Awad El-Sayed Z, Bacharier LB, Bonini M, Braido F, Caimmi D, Castro-Rodriguez JA, Chen Z, Clausen M, Craig T, Diamant Z, Ducharme FM, Ebisawa M, Eigenmann P, Feleszko W, Fierro V, Fiocchi A, Garcia-Marcos L, Goh A, Gómez RM, Gotua M, Hamelmann E, Hedlin G, Hossny EM, Ispayeva Z, Jackson DJ, Jartti T, Jeseňák M, Kalayci O, Kaplan A, Konradsen JR, Kuna P, Lau S, Le Souef P, Lemanske RF, Levin M, Makela MJ, Mathioudakis AG, Mazulov O, Morais-Almeida M, Murray C, Nagaraju K, Novak Z, Pawankar R, Pijnenburg MW, Pite H, Pitrez PM, Pohunek P, Price D, Priftanji A, Ramiconi V, Rivero Yeverino D, Roberts G, Sheikh A, Shen KL, Szepfalusi Z, Tsiligianni I, Turkalj M, Turner S, Umanets T, Valiulis A, Vijveberg S, Wang JY, Winders T, Yon DK, Yusuf OM, Zar HJ; WAO Pediatric Asthma Committee.. Pediatr Allergy Immunol. 2024 Apr;35(4):e14129. doi: 10.1111/pai.14129. Erratum in: Pediatr Allergy Immunol. 2024 Dec;35(12):e70005. doi: 10.1111/pai.70005. PMID: 38664926 : Recommendations for asthma monitoring in children: A PeARL document endorsed by APAPARI, EAACI, INTERASMA, REG, and WAO – PubMed
Short- and long-term clinical outcomes of nintedanib therapy in IPF patients with different phenotypes: A retrospective registry-based study
Authors: Koziar Vašáková M, Gregor J, Mogulkoc N, Kramer MR, Lewandowska K, Šterclová M, Müller V, Slivka R, Studnicka M, Plačková M, Žurková M, Tekavec-Trkanjec J, Doubková M, Ovesná P; EMPIRE investigators.. Respir Med. 2024 Nov-Dec;234:107791. doi: 10.1016/j.rmed.2024.107791. Epub 2024 Sep 8. PMID: 39255912. https://pubmed.ncbi.nlm.nih.gov/39255912/
The Impact of Switching to a Second Antifibrotic in Patients With Idiopathic Pulmonary Fibrosis: A Retrospective Multicentre Study From the EMPIRE Registry
Authors: Gregor J, Adir Y, Šterclová M, Mogulkoc N, Kramer MR, Doubková M, Plačková M, Müller V, Studnicka M, Žurková M, Lacina L, Lewandowska K, Bartoš V, Ovesná P, Májek O, Koziar Vašáková M.. Arch Bronconeumol. 2024 Feb;60(2):80-87. English, Spanish. doi: 10.1016/j.arbres.2023.12.002. Epub 2023 Dec 18. PMID: 38160169. https://pubmed.ncbi.nlm.nih.gov/38160169/
Treatable traits in idiopathic pulmonary fibrosis: focus on respiratory tract infections-a systematic review and a meta-analysis
Authors: Matics Z, Bardóczi A, Galkó C, Szabó B, Gede N, Molnár Z, Duray G, Turan C, Hegyi P, Horváth G, Müller V.. EClinicalMedicine. 2024 Dec 5;79:102966. doi: 10.1016/j.eclinm.2024.102966. PMID: 39720602; PMCID: PMC11665700. https://pubmed.ncbi.nlm.nih.gov/39720602/
“Alveolar stem cell exhaustion, fibrosis and bronchiolar proliferation” related entities. A narrative review.
Authors: Chilosi, M; Piciucchi, S; Ravaglia, C; Spagnolo, P; Sverzellati, N; Tomassetti, S; Wuyts, W; Poletti, V (2024). PULMONOLOGY, pp. 0-0, ISSN:2531-0437 DOI
The Performance of Pulmonary Function Tests in Predicting Systemic Sclerosis-Interstitial Lung Disease in the European Scleroderma Trial and Research Database.
Authors: Gemma Lepri, Cosimo Bruni, Lorenzo Tofani, Alberto Moggi-Pignone, Martina Orlandi, Sara Tomassetti, Michael Hughes, Francesco Del Galdo, Rosaria Irace, Oliver Distler, Valeria Riccieri, Yannick Allanore, Ana Maria Gheorghiu, Elise Siegert, Jeska De Vries-Bouwstra, Eric Hachulla, Mohammed Tikly, Nemanja Damjanov, Francois Spertini, Luc Mouthon, Anna-Maria Hoffmann-Vold, Armando Gabrielli, Serena Guiducci, Marco Matucci-Cerinic, Daniel Furst, Silvia Bellando-Randone (2024). DIAGNOSTICS, vol. 14, pp. 1-14, ISSN:2075-4418 DOI
The role of lung biopsy for diagnosis and prognosis of interstitial lung disease in systemic sclerosis: a systematic literature review.
Authors: Damiani, A; Orlandi, M; Bruni, C; Bandini, G; Lepri, G; Scaletti, C; Ravaglia, C; Frassanito, F; Guiducci, S; Moggi-Pignone, A; Matucci-Cerinic, M; Poletti, V; Tofani, L; Colby, TV; Randone, S Bellando; Tomassetti, Sara (2024). RESPIRATORY RESEARCH, vol. 25, pp. 0-0, ISSN:1465-993X DOI
Presentation and progression of MPO-ANCA interstitial lung disease.
Authors: Salvati L.; Palterer B.; Lazzeri E.; Vivarelli E.; Amendola M.; Allinovi M.; Caroti L.; Mazzoni A.; Lasagni L.; Emmi G.; Cavigli E.; Del Carria M.; Di Pietro L.; Scavone M.; Cammelli D.; Lavorini F.; Tomassetti S.; Rosi E.; Parronchi P. (2024 JOURNAL OF TRANSLATIONAL AUTOIMMUNITY, vol. 8, pp. 0-0, ISSN:2589-9090
Identification of progressive pulmonary fibrosis: consensus findings from a modified Delphi study.
Authors: Wells AU, Walsh SLF, Adegunsoye A, Cottin V, Danoff SK, Devaraj A, Flaherty KR, George PM, Johannson KA, Kolb M, Kondoh Y, Nicholson AG, Tomassetti S, Volkmann ER, Brown KK.Respir Res. 2024 Dec 31;25(1):448. doi: 10.1186/s12931-024-03070-z.PMID: 39741294
Breaking barriers: holistic assessment of ability to work in patients with sarcoidosis.
Authors: Drent M, Russell AM, Saketkoo LA, Spagnolo P, Veltkamp M, Wells AU; representatives of the sarcoidosis community..Lancet Respir Med. 2024 Nov;12(11):848-851. doi: 10.1016/S2213-2600(24)00297-2. Epub 2024 Oct 16.PMID: 39423840
Perioperative Anti-Fibrotic Treatment Prevents Acute Exacerbation of Idiopathic Pulmonary Fibrosis After Lung Cancer Surgery.
Authors: Bongiolatti S, Salvicchi A, Rosi E, Bargagli E, Mugnaini G, Gonfiotti A, Lavorini F, Spagnolo P, Dell’Amore A, Rea F, Voltolini L. Life (Basel). 2024 Nov 19;14(11):1506. doi: 10.3390/life14111506. PMID: 39598304
Mortality trends in idiopathic pulmonary fibrosis in Europe between 2013 and 2018.
Authors: Gonnelli F, Bonifazi M, Hubbard R Eur Respir J. 2024 Aug 22;64(2):2302080.
Pulmonary fibrosis and lung cancer: an analysis of the Clinical Practice Research Datalink linked to the National Cancer Registration Dataset.
Authors: Gonnelli F, Kaur J, Bonifazi M, Baldwin D, O’Dowd E, Hubbard R. Thorax. 2024 Sep 18;79(10):982-985.
Incidence and survival of interstitial lung diseases in the UK in 2010-2019.
Authors: Gonnelli F, Eleangovan N, Smith U, Heatley H, Navarantam V, Corte TJ, Price DB, Carter V, Bonifazi M, Fermoyle CC, Hubbard R. ERJ Open Res. 2025 Mar 3;11(2):00823-2024.
Pulmonary lymphangiomatosis: insights into an ultra-rare disease.
Authors: Polke M, Polke N, Piel S, Brunnemer E, Wälscher J, Buschulte K, Warth A, Heussel CP, Eichinger M, Frankenstein L, Eichhorn M, Miliauskas S, Herth FJF, Kreuter M. Respir Res. 2024 Nov 26;25(1):416. doi: 10.1186/s12931-024-03040-5. PMID: 39593123; PMCID: PMC11600747
Practical guidance for the early recognition and follow-up of patients with connective tissue disease-related interstitial lung disease.
Authors: Guiot J, Miedema J, Cordeiro A, De Vries-Bouwstra JK, Dimitroulas T, Søndergaard K, Tzouvelekis A, Smith V. Autoimmun Rev. 2024 Jun;23(6):103582. doi: 10.1016/j.autrev.2024.103582. Epub 2024 Jul 27. Erratum in: Autoimmun Rev. 2024 Oct;23(10):103641. doi: 10.1016/j.autrev.2024.103641. PMID: 39074630.
Clinical implications of nintedanib pharmacokinetics in patients with pulmonary fibrosis.
Authors: Agema BC, Berrich M, Seuren L, Sassen SDT, Miedema JR, Koch BCP, Wijsenbeek MS, Koolen SLW, Mathijssen RHJ, Veerman GDM. Biomed Pharmacother. 2024 Oct;179:117341. doi: 10.1016/j.biopha.2024.117341. Epub 2024 Aug 26. PMID: 39191023.
Transbronchial cryobiopsy followed by as-needed surgical lung biopsy versus immediate surgical lung biopsy for diagnosing interstitial lung disease (the COLD study): a randomised controlled trial.
Authors: Kalverda KA, Ninaber MK, Wijmans L, von der Thüsen J, Jonkers RE, Daniels JM, Miedema JR, Dickhoff C, Hölters J, Heineman D, Kant M, Radonic T, Shahin G, Cohen D, Boerrigter B, Nijman S, Nossent E, Braun J, Mathot B, Poletti V, Hetzel J, Dijkgraaf M, Korevaar DA, Bonta PI, Annema JT. Lancet Respir Med. 2024 Jul;12(7):513-522. doi: 10.1016/S2213-2600(24)00074-2. Epub 2024 Apr 16. PMID: 38640934.
A modified Delphi exercise in physician-perceived risk factors for drug-induced pneumotoxicity in patients with rheumatological disease.
Authors: Cartlidge MK, Brown KK, Chaudhuri N, Corte TJ, Dieudé P, John L, Kelly C, Khanna D, McRorie E, Nicol L, Stewart G, Walsh SLF, Wijsenbeek M, Hirani N; PASSWRD participants. BMC Pulm Med. 2024 Oct 31;24(1):547. doi: 10.1186/s12890-024-03287-0. PMID: 39482644; PMCID: PMC11529426.
Patient-centered care in pulmonary fibrosis: access, anticipate, and act.
Authors: Hofman DE, Magrì T, Moor CC, Richeldi L, Wijsenbeek MS, Waseda Y.. Respir Res. 2024 Nov 1;25(1):395. doi: 10.1186/s12931-024-02997-7. PMID: 39487454; PMCID: PMC11531140.
Breathing techniques to reduce symptoms in people with serious respiratory illness: a systematic review.
Authors: Burge AT, Gadowski AM, Jones A, Romero L, Smallwood NE, Ekström M, Reinke LF, Saggu R, Wijsenbeek M, Holland AE. Eur Respir Rev. 2024 Oct 30;33(174):240012. doi: 10.1183/16000617.0012-2024. PMID: 39477355; PMCID: PMC11522968.
Opioids for the palliation of symptoms in people with serious respiratory illness: a systematic review and meta-analysis.
Authors: Smallwood NE, Pascoe A, Wijsenbeek M, Russell AM, Holland AE, Romero L, Ekström M. Eur Respir Rev. 2024 Oct 9;33(174):230265. doi: 10.1183/16000617.0265-2023. PMID: 39384304; PMCID: PMC11462312.
Objective measurement of cough in pulmonary fibrosis: a cohort study – ImpaCT.
Authors: Kebbe J, Hart SP, Kaner RJ, Kulkarni T, Wollin L, Coeck C, Vinisko R, Patel N, Wijsenbeek MSERJ Open Res. 2024 Sep 30;10(5):00310-2024. doi: 10.1183/23120541.00310-2024. PMID: 39351380; PMCID: PMC11440380.
Is YouTube a sufficient source of information on Sarcoidosis?
Authors: Buschulte K, El-Hadi S, Höger P, Ganter C, Wijsenbeek M, Kahn N, Kriegsmann K, Goobie GC, Ryerson CJ, Polke M, Trudzinski F, Kreuter MRespir Res. 2024 Sep 9;25(1):334. doi: 10.1186/s12931-024-02956-2. PMID: 39252025; PMCID: PMC11386059.
INBUILD trial investigators. Worsening dyspnoea as a predictor of progression of pulmonary fibrosis.
Authors: Wijsenbeek MS, Swigris JJ, Spagnolo P, Kolb M, Kreuter M, Nunes H, Stansen W, Rohr KB, Inoue Y; Eur Respir J. 2024 Oct 31;64(4):2302211. doi: 10.1183/13993003.02211-2023. PMID: 39227075; PMCID: PMC11525330.
The burden of cough in idiopathic pulmonary fibrosis and other interstitial lung diseases: a systematic evidence synthesis.
Authors: Green R, Baldwin M, Pooley N, Misso K, Mölken MPR, Patel N, Wijsenbeek MS. Respir Res. 2024 Aug 27;25(1):325. doi: 10.1186/s12931-024-02897-w. PMID: 39192278; PMCID: PMC11351049.
Integrating the assessment of quality of life in care and research in pulmonary fibrosis.
Authors: Aronson KI, Swigris JJ, Wijsenbeek M. Curr Opin Pulm Med. 2024 Sep 1;30(5):508-515. doi: 10.1097/MCP.0000000000001095. Epub 2024 Jul 1. PMID: 38946547.
Detection of Systemic Sclerosis- associated Interstitial Lung Disease by Exhaled Breath Analysis Using Electronic Nose Technology.
Authors: Marges ER, van der Sar IG, de Vries-Bouwstra JK, Huizinga TWJ, van Daele PLA, Wijsenbeek MS, Moor CC, Geelhoed JJM. Am J Respir Crit Care Med. 2024 Aug 15;210(4):512-514. doi: 10.1164/rccm.202402-0272LE. PMID: 38843104.
Classifying Interstitial Lung Disease: Omics Are in the Air.
Authors: van der Sar IG, Moor CC, Wijsenbeek MS. Am J Respir Crit Care Med. 2024 Sep 1;210(5):690-691. doi: 10.1164/rccm.202404-0748LE. PMID: 38701488; PMCID: PMC11389585.
Detection of Drug-induced Interstitial Lung Disease Caused by Cancer Treatment Using Electronic Nose Exhaled Breath Analysis.
Authors: van der Sar IG, Wijsenbeek MS, Dumoulin DW, Jager A, van der Veldt AAM, Rossius MJP, Dingemans AC, Moor CC. Ann Am Thorac Soc. 2024 Jun;21(6):989-992. doi: 10.1513/AnnalsATS.202401-112RL. PMID: 38526585.
Morphine for treatment of cough in idiopathic pulmonary fibrosis (PACIFY COUGH): a prospective, multicentre, randomised, double-blind, placebo- controlled, two-way crossover trial.
Authors: Wu Z, Spencer LG, Banya W, Westoby J, Tudor VA, Rivera-Ortega P, Chaudhuri N, Jakupovic I, Patel B, Thillai M, West A, Wijsenbeek M, Maher TM, Smith JA, Molyneaux PL. Lancet Respir Med. 2024 Apr;12(4):273-280. doi: 10.1016/S2213-2600(23)00432-0. Epub 2024 Jan 15. PMID: 38237620.
Meaningful Endpoints for Idiopathic Pulmonary Fibrosis (IPF) Clinical Trials: Emphasis on ‘Feels, Functions, Survives’. Report of a Collaborative Discussion in a Symposium with Direct Engagement from Representatives of Patients, Investigators, the National Institutes of Health, a Patient Advocacy Organization, and a Regulatory Agency.
Authors: Wijsenbeek M et al. Am J Respir Crit Care Med. 2024 Mar 15;209(6):647-669. doi: 10.1164/rccm.202312-2213SO. PMID: 38174955.
Effects of nintedanib on symptoms in patients with progressive pulmonary fibrosis.
Authors: Wijsenbeek M, Swigris JJ, Inoue Y, Kreuter M, Maher TM, Suda T, Baldwin M, Mueller H, Rohr KB, Flaherty KR; INBUILD Trial InvestigatorsEur Respir J. 2024 Feb 1;63(2):2300752. doi: 10.1183/13993003.00752-2023. PMID: 38135442; PMCID: PMC10831140.
Sexual Dysfunction in Patients With Sarcoidosis.
Authors: Moor CC, Gur-Demirel Y, Koudstaal T, Miedema JR. Sexual Chest. 2024 Dec;166(6):1473-1475. doi: 10.1016/j.chest.2024.07.159. Epub 2024 Aug 10. PMID: 39134142.
The immunopathogenesis of sarcoidosis.
Authors: Miedema J, Cinetto F, Smed-Sörensen A, Spagnolo P. J Autoimmun. 2024 Dec;149:103247. doi: 10.1016/j.jaut.2024.103247. Epub 2024 May 10. PMID: 38734536.
Quality of life in sarcoidosis.
Authors: Moor CC, Obi ON, Kahlmann V, Buschulte K, Wijsenbeek MS. J Autoimmun. 2024 Dec;149:103123. doi: 10.1016/j.jaut.2023.103123. Epub 2023 Oct 8. PMID: 37813805.
Circulating T cells in sarcoidosis have an aberrantly activated phenotype that correlates with disease outcome.
Authors: Miedema JR, de Jong LJ, van Uden D, Bergen IM, Kool M, Broos CE, Kahlmann V, Wijsenbeek MS, Hendriks RW, Corneth OBJ. J Autoimmun. 2024 Dec;149:103120. doi: 10.1016/j.jaut.2023.103120. Epub 2023 Oct 18. PMID: 37863732.
Increased proportions of circulating PD-1+ CD4+ memory T cells and PD-1+ regulatory T cells associate with good response to prednisone in pulmonary sarcoidosis.
Authors: Miedema JR, de Jong LJ, Kahlmann V, Bergen IM, Broos CE, Wijsenbeek MS, Hendriks RW, Corneth OBJRespir Res. 2024 May 7;25(1):196. doi: 10.1186/s12931-024-02833-y. PMID: 38715030; PMCID: PMC11075187.
The developing immune system in preterm born infants: From contributor to potential solution for respiratory tract infections and wheezing.
Authors: Duijts L et al. Allergy. 2024 Nov;79(11):2924-2942. doi: 10.1111/all.16342.
2023
Updated Prevalence of Lymphangioleiomyomatosis in Europe.
Authors: Lynn E, Forde SH, Franciosi AN, Bendstrup E, Veltkamp M, Wind AE, Van Moorsel CHM, Lund TK, Durheim MT, Peeters EFHI, Keane MP, McCarthy C. . Am J Respir Crit Care Med. 2023 Dec 7. doi: 10.1164/rccm.202310-1736LE. Epub ahead of print. PMID: 38060201.2. 17.
Idiopathic Pulmonary Fibrosis Is Associated with Common Genetic Variants and Limited Rare Variants.
Authors: Peljto AL, Blumhagen RZ, Walts AD, Cardwell J, Powers J, Corte TJ, Dickinson JL, Glaspole I, Moodley YP, Vasakova MK, Bendstrup E, Davidsen JR, Borie R, Crestani B, Dieude P, Bonella F, Costabel U, Gudmundsson G, Donnelly SC, Egan J, Henry MT, Keane MP, Kennedy MP, McCarthy C, McElroy AN, Olaniyi JA, O’Reilly KMA, Richeldi L, Leone PM, Poletti V, Puppo F, Tomassetti S, Luzzi V, Kokturk N, Mogulkoc N, Fiddler CA, Hirani N, Jenkins G, Maher TM, Molyneaux PL, Parfrey H, Braybrooke R, Blackwell TS, Jackson PD, Nathan SD, Porteous MK, Brown KK, Christie JD, Collard HR, Eickelberg O, Foster EE, Gibson KF, Glassberg M, Kass D, Kropski JA, Lederer D, Linderholm AL, Loyd J, Mathai SK, Montesi SB, Noth I, Oldham JM, Palmisciano AJ, Reichner CA, Rojas M, Roman J, Schluger N, Shea BS, Swigris JJ, Wolters PJ, Zhang Y, Prele CMA, Enghelmayer JI, Otaola M, Ryerson CJ, Salinas M, Sterclova M, Gebremariam TH, Myllärniemi M, Carbone R, Furusawa H, Hirose M, Inoue Y, Miyazaki Y, Ohta K, Ohta S, Okamoto T, Kim DS, Pardo A, Selman M, Aranda AU, Park MS, Park JS, Song JW, Molina-Molina M, Planas-Cerezales L, Westergren-Thorsson G, Smith AV, Manichaikul AW, Kim JS, Rich SS, Oelsner EC, Barr RG, Rotter JI, Dupuis J, O’Connor G, Vasan RS, Cho MH, Silverman EK, Schwarz MI, Steele MP, Lee JS, Yang IV, Fingerlin TE, Schwartz DA; NHLBI Trans-Omics for Precision Medicine (TOPMed) Consortium. Am J Respir Crit Care Med. 2023 Jan 5. doi: 10.1164/rccm.202207-1331OC. Epub ahead of print. PMID: 36602845.
Diagnostic workup of childhood interstitial lung disease.
Authors: Nathan N, Griese M, Michel K, Carlens J, Gilbert C, Emiralioglu N, Torrent-Vernetta A, Marczak H, Willemse B, Delestrain C, Epaud R; ERS CRC chILD-EU group. Eur Respir Rev. 2023 Feb 21;32(167):220188.2.
ABCA3-related interstitial lung disease beyond infancy.
Authors: Li Y, Seidl E, Knoflach K, Gothe F, Forstner ME, Michel K, Pawlita I, Gesenhues F, Sattler F, Yang X, Kroener C, Reu-Hofer S, Ley-Zaporozhan J, Kammer B, Krüger-Stollfuß I, Dinkel J, Carlens J, Wetzke M, Moreno-Galdó A, Torrent-Vernetta A, Lange J, Krenke K, Rumman N, Mayell S, Sismanlar T, Aslan A, Regamey N, Proesmans M, Stehling F, Naehrlich L, Ayse K, Becker S, Koerner-Rettberg C, Plattner E, Manali ED, Papiris SA, Campo I, Kappler M, Schwerk N, Griese M. Thorax. 2023 Jun;78(6):587-595
BI 1015550: an investigational phosphodiesterase 4B (PDE4B) inhibitor for lung function decline in idiopathic pulmonary fibrosis (IPF).
Authors: Sgalla G, Simonetti J, Cortese S, Richeldi L. Expert Opin Investig Drugs. 2023 Jan;32(1):17-23. doi: 10.1080/13543784.2023.2173061. Epub 2023 Feb 3. PMID: 36693635 Review.
Ziritaxestat, a Novel Autotaxin Inhibitor, and Lung Function in Idiopathic Pulmonary Fibrosis: The ISABELA 1 and 2 Randomized Clinical Trials.
Authors: Maher TM, Ford P, Brown KK, Costabel U, Cottin V, Danoff SK, Groenveld I, Helmer E, Jenkins RG, Milner J, Molenberghs G, Penninckx B, Randall MJ, Van Den Blink B, Fieuw A, Vandenrijn C, Rocak S, Seghers I, Shao L, Taneja A, Jentsch G, Watkins TR, Wuyts WA, Kreuter M, Verbruggen N, Prasad N, Wijsenbeek MS; ISABELA 1 and 2 Investigators. JAMA. 2023 May 9;329(18):1567-1578. doi: 10.1001/jama.2023.5355. PMID: 37159034 Free PMC article.
Design of a phase III, double-blind, randomised, placebo-controlled trial of BI 1015550 in patients with idiopathic pulmonary fibrosis (FIBRONEER-IPF).
Authors: Richeldi L, Azuma A, Cottin V, Kreuter M, Maher TM, Martinez FJ, Oldham JM, Valenzuela C, Gordat M, Liu Y, Stowasser S, Zoz DF, Wijsenbeek MS. BMJ Open Respir Res. 2023 Aug;10(1):e001563. doi: 10.1136/bmjresp-2022-001563. PMID: 37597969 Free PMC article. Clinical Trial.
The Future of Clinical Trials in Idiopathic Pulmonary Fibrosis.
Authors: Podolanczuk AJ, Richeldi L, Martinez FJ. JAMA. 2023 May 9;329(18):1554-1555. doi: 10.1001/jama.2022.23955. PMID: 37159046 No abstract available.
An update on emerging drugs for the treatment of idiopathic pulmonary fibrosis: a look towards 2023 and beyond.
Authors: Sofia C, Comes A, Sgalla G, Richeldi L. Expert Opin Emerg Drugs. 2023 Dec;28(4):283-296. doi: 10.1080/14728214.2023.2281416. Epub 2023 Dec 26. PMID: 37953604 Review.
An up-to-date review of approved and emerging antibody therapies for idiopathic pulmonary fibrosis.
Authors: Simonetti J, Sgalla G, Richeldi L. Expert Opin Biol Ther. 2023 Jul-Dec;23(12):1239-1244. doi:10.1080/14712598.2023.2268014. Epub 2023 Dec 28. PMID: 37797203 Review.
Challenges in the diagnosis of idiopathic pulmonary fibrosis: the importance of a multidisciplinary approach.
Authors: Comes A, Sgalla G, Ielo S, Magrì T, Richeldi L. Expert Rev Respir Med. 2023 Apr;17(4):1-11. doi: 10.1080/17476348.2023.2199156. Epub 2023 Apr 26. PMID: 37005289 Review. Page 2
Approach to Clinical Trials for the Prevention of Pulmonary Fibrosis.
Authors: Kim JS, Montesi SB, Adegunsoye A, Humphries SM, Salisbury ML, Hariri LP, Kropski JA, Richeldi L, Wells AU, Walsh S, Jenkins RG, Rosas I, Noth I, Hunninghake GM, Martinez FJ, Podolanczuk AJ. Ann Am Thorac Soc. 2023 Dec;20(12):1683-1693. doi: 10.1513/AnnalsATS.202303-188PS. PMID: 37703509 No abstract available.
GATA1-defective immune-megakaryocytes as possible drivers of idiopathic pulmonary fibrosis.
Authors: Gobbo F, Zingariello M, Verachi P, Falchi M, Arciprete F, Martelli F, Peli A, Mazzarini M, Vierstra J, Mead-Harvey C, Dueck AC, Sarli G, Nava S, Sgalla G, Richeldi L, Migliaccio AR. bioRxiv [Preprint]. 2023 Sep 19:2023.06.20.542249. doi: 10.1101/2023.06.20.542249. PMID: 37425686 Free PMC article. Preprint.
Design of a phase III, double-blind, randomised, placebo-controlled trial of BI 1015550 in patients with progressive pulmonary fibrosis (FIBRONEER-ILD).
Authors: Maher TM, Assassi S, Azuma A, Cottin V, Hoffmann-Vold AM, Kreuter M, Oldham JM, Richeldi L, Valenzuela C, Wijsenbeek MS, Coeck C, Schlecker C, Voss F, Wachtlin D, Martinez FJ. BMJ Open Respir Res. 2023 Sep;10(1):e001580. doi: 10.1136/bmjresp-2022-001580. PMID: 37709661 Free PMC article. Clinical Trial.
Summary for Clinicians: Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults.
Authors: Herman DD, Ghazipura M, Raghu G, Richeldi L, Remy-Jardin M, Ruminjo JK, Thomson CC. Ann Am Thorac Soc. 2023 May;20(5):632-637. doi: 10.1513/AnnalsATS.202211-924CME. PMID: 36746177 No abstract available.
Observational, Multicenter Study on the Efficacy, Tolerability, and Safety of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis Older than 80 Years.
Authors: Mondoni M, Alfano F, Varone F, Muscato G, Conti C, Saderi L, Chiesa A, Di Marco F, Vancheri C, Richeldi L, Centanni S, Sotgiu G. Respiration. 2023;102(1):25-33. doi: 10.1159/000527308. Epub 2022 Nov 10. PMID: 36366821 Free article. Reply to Cottin et al., to Johannson et al., to Scholand and Wells, and to Crowley et al. Raghu G, Richeldi L, Thomson CC, Remy-Jardin M, Wilson KC. Am J Respir Crit Care Med. 2023 Jun 1;207(11):1542-1543. doi: 10.1164/rccm.202305-0786LE. PMID: 37130124 Free PMC article. No abstract available.
Predictors of Stability/Improvement of Forced Vital Capacity in Patients With Idiopathic Pulmonary Fibrosis After One Year of Treatment With Nintedanib. Authors: Mondoni M, Varone F, Alfano F, Muscato G, Conti C, Saderi L, Iovene B, Marco FD, Vancheri C, Richeldi L, Centanni S, Sotgiu G. Arch Bronconeumol. 2023 Jul;59(7):464-466. doi: 10.1016/j.arbres.2023.02.010. Epub 2023 Feb 27. PMID: 36894471 Free article.
Monitoring small airway dysfunction in connective tissue disease-related interstitial lung disease: a retrospective and prospective study.
Authors: Xu L, Sgalla G, Wang F, Zhu M, Li L, Li P, Xie Q, Lv X, Yu J, Wang G, Wan H, Richeldi L, Luo F. BMC Pulm Med. 2023 Mar 20;23(1):90. doi: 10.1186/s12890-023-02381-z. PMID: 36941622 Free PMC article. Page 3
Treatable traits in interstitial lung diseases: a call to action.
Authors: Amati F, Spagnolo P, Oldham JM, Ryerson CJ, Stainer A, Gramegna A, Mantero M, Lacedonia D, Sverzellati N, Richeldi L, Blasi F, Aliberti S. Lancet Respir Med. 2023 Feb;11(2):125-128. doi: 10.1016/S2213-2600(23)00002-4. Epub 2023 Jan 13. PMID: 36646100 No abstract available.
Walking the path of treatable traits in interstitial lung diseases.
Authors: Amati F, Spagnolo P, Ryerson CJ, Oldham JM, Gramegna A, Stainer A, Mantero M, Sverzellati N, Lacedonia D, Richeldi L, Blasi F, Aliberti S. Respir Res. 2023 Oct 24;24(1):251. doi: 10.1186/s12931-023-02554-8. PMID: 37872563 Free PMC article.
European Respiratory Society statement on familial pulmonary fibrosis.
Authors: Borie R, Kannengiesser C, Antoniou K, Bonella F, Crestani B, Fabre A, Froidure A, Galvin L, Griese M, Grutters JC, Molina-Molina M, Poletti V, Prasse A, Renzoni E, van der Smagt J, van Moorsel CHM. Eur Respir J. 2023 Mar 16;61(3):2201383. doi: 10.1183/13993003.01383-2022. PMID: 36549714.
Current and Future Treatment Landscape for Idiopathic Pulmonary Fibrosis.
Authors: Bonella F, Spagnolo P, Ryerson C. Drugs. 2023 Nov;83(17):1581-1593. doi: 10.1007/s40265-023-01950-0. Epub 2023 Oct 26. PMID: 37882943; PMCID: PMC10693523.
Pulmonary Fibrosis Is Associated with Common Genetic Variants and Limited Rare Variants.
Authors: Peljto AL, Blumhagen RZ, Walts AD, Cardwell J, Powers J, Corte TJ, Dickinson JL, Glaspole I, Moodley YP, Vasakova MK, Bendstrup E, Davidsen JR, Borie R, Crestani B, Dieude P, Bonella F, Costabel U, Gudmundsson G, Donnelly SC, Egan J, Henry MT, Keane MP, Kennedy MP, McCarthy C, McElroy AN, Olaniyi JA, O’Reilly KMA, Richeldi L, Leone PM, Poletti V, Puppo F, Tomassetti S, Luzzi V, Kokturk N, Mogulkoc N, Fiddler CA, Hirani N, Jenkins RG, Maher TM, Molyneaux PL, Parfrey H, Braybrooke R, Blackwell TS, Jackson PD, Nathan SD, Porteous MK, Brown KK, Christie JD, Collard HR, Eickelberg O, Foster EE, Gibson KF, Glassberg M, Kass DJ, Kropski JA, Lederer D, Linderholm AL, Loyd J, Mathai SK, Montesi SB, Noth I, Oldham JM, Palmisciano AJ, Reichner CA, Rojas M, Roman J, Schluger N, Shea BS, Swigris JJ, Wolters PJ, Zhang Y, Prele CMA, Enghelmayer JI, Otaola M, Ryerson CJ, Salinas M, Sterclova M, Gebremariam TH, Myllärniemi M, Carbone RG, Furusawa H, Hirose M, Inoue Y, Miyazaki Y, Ohta K, Ohta S, Okamoto T, Kim DS, Pardo A, Selman M, Aranda AU, Park MS, Park JS, Song JW, Molina-Molina M, Planas- Cerezales L, Westergren-Thorsson G, Smith AV, Manichaikul AW, Kim JS, Rich SS, Oelsner EC, Barr RG, Rotter JI, Dupuis J, O’Connor G, Vasan RS, Cho MH, Silverman EK, Schwarz MI, Steele MP, Lee JS, Yang IV, Fingerlin TE, Schwartz DA. Idiopathic Am J Respir Crit Care Med. 2023 May 1;207(9):1194-1202. doi: 10.1164/rccm.202207-1331OC. Erratum in: Am J Respir Crit Care Med. 2024 Feb 15;209(4):462. PMID: 36602845; PMCID: PMC10161752.
Pulmonary alveolar proteinosis – current and future therapeutical strategies.
Authors: Jehn LB, Bonella F. Curr Opin Pulm Med. 2023 Sep 1;29(5):465-474. doi: 10.1097/MCP.0000000000000982. Epub 2023 Jun 28. PMID: 37395514.
Gastrointestinal pirfenidone adverse events in idiopathic pulmonary fibrosis depending on diet: the MADIET clinical trial.
Authors: Molina-Molina M, Shull JG, Vicens-Zygmunt V, Rivera-Ortega P, Antoniou K, Bonella F, Renzoni E, Russell AM, Maher TM, Vancheri A, Bachs A, Avilés V, Palma J, Bermudo G, Suarez-Cuartin G, Tebé C, Rigo-Bonnin R, Montes-Worboys A, Wijsenbeek M, Vancheri C. Eur Respir J. 2023 Oct 19;62(4):2300262. doi: 10.1183/13993003.00262-2023. PMID: 37857429; PMCID: PMC10586233.
COVID-19 in patients with pulmonary alveolar proteinosis: a European multicentre study.
Authors: Papiris SA, Campo I, Mariani F, Kallieri M, Kolilekas L, Papaioannou AI, Gonca Chousein E, Cetinkaya E, Bonella F, Borie R, Kokosi M, Pickworth T, Molina-Molina M, Gasa M, Radzikowska E, Fijolek J, Jouneau S, Gomez E, McCarthy C, Bendstrup E, Piotrowski WJ, Pabary R, Hadchouel A, Coolen-Allou N, Alfaro T, Robalo Cordeiro C, Antonogiannaki EM, Tomos IP, Papakosta D, Kontakiotis T, Panagiotou P, Douros K, Schams A, Lettieri S, Papaevangelou V, Kanaka-Gantenbein C, Karakatsani A, Loukides S, Costabel U, Crestani B, Morgan C, Tazawa R, Bush A, Griese M, Manali ED. ERJ Open Res. 2023 Jan 3;9(1):00199-2022. doi: 10.1183/23120541.00199-2022. PMID: 36601310; PMCID: PMC9271262.
S1-Leitlinie Interdisziplinäre Diagnostik interstitieller Lungenerkrankungen im Erwachsenenalter [Consensus guideline on the interdisciplinary diagnosis of interstitial lung diseases].
Authors: Kreuter M, Behr J, Bonella F, Costabel U, Gerber A, Hamer OW, Heussel CP, Jonigk D, Krause A, Koschel D, Leuschner G, Markart P, Nowak D, Pfeifer M, Prasse A, Wälscher J, Winter H, Kabitz HJ. Pneumologie. 2023 May;77(5):269-302. German. doi: 10.1055/a-2017-8971. Epub 2023 Mar 28. PMID: 36977470.
MUC5B rs35705950 minor allele associates with older age and better survival in idiopathic pulmonary fibrosis.
Authors: van der Vis JJ, Prasse A, Renzoni EA, Stock CJW, Caliskan C, Maher TM, Bonella F, Borie R, Crestani B, Petrek M, Wuyts WA, Wind AE, Molyneaux PL, Grutters JC, van Moorsel CHM. Respirology. 2023 May;28(5):455-464. doi: 10.1111/resp.14440. Epub 2022 Dec 26. PMID: 36571111.
Lung cancer in patients with idiopathic pulmonary fibrosis: A retrospective multicentre study in Europe.
Authors: Karampitsakos T, Spagnolo P, Mogulkoc N, Wuyts WA, Tomassetti S, Bendstrup E, Molina-Molina M, Manali ED, Unat ÖS, Bonella F, Kahn N, Kolilekas L, Rosi E, Gori L, Ravaglia C, Poletti V, Daniil Z, Prior TS, Papanikolaou IC, Aso S, Tryfon S, Papakosta D, Tzilas V, Balestro E, Papiris S, Antoniou K, Bouros D, Wells A, Kreuter M, Tzouvelekis A. Respirology. 2023 Jan;28(1):56-65. doi: 10.1111/resp.14363. Epub 2022 Sep 18. PMID: 36117239.
Historical eye on IPF: a cohort study redefining the mortality scenario.
Authors: Tomassetti S, Ravaglia C, Piciucchi S, Ryu J, Wells A, Donati L, Dubini A, Klersy C, Luzzi V, Gori L, Rosi E, Lavorini F, Poletti V. Front Med (Lausanne). 2023 Jun 2;10:1151922. doi: 10.3389/fmed.2023.1151922. PMID: 37332746; PMCID: PMC10273674
Clinical implications of interstitial pneumonia with autoimmune features diagnostic criteria in idiopathic pulmonary fibrosis: A case control study.
Authors: Tomassetti S, Ravaglia C, Puglisi S, Wells AU, Ryu JH, Bosi M, Dubini A, Piciucchi S, Girelli F, Parronchi P, Lavorini F, Rosi E, Luzzi V, Cerinic MM, Poletti V. Front Med (Lausanne). 2023 Feb 16;10:1087485. doi: 10.3389/fmed.2023.1087485. PMID: 36873871; PMCID: PMC9978138.
Update of penetrance estimates in Birt-Hogg-Dubé syndrome.
Authors: Bruinsma FJ, Dowty JG, Win AK, Goddard LC, Agrawal P, Attina’ D, Bissada N,De Luise M, Eisen DB, Furuya M, Gasparre G, Genuardi M, Gerdes AM,Hansen TVO,Houweling AC, Johannesma PC, Lencastre A, Lim D, Lindor NM, Luzzi V,Lynch M,Maffé A, Menko FH, Michels G, Pulido JS, Ryu JH, Sattler EC, Steinlein OK,Tomassetti S, Tucker K, Turchetti D, van de Beek I, van Riel L, van Steensel M,Zenone T, Zompatori M, Walsh J, Bondavalli D, Maher ER, Winship IM; Genetic Susceptibility Working Group I-CONFIRM. J Med Genet. 2023 Apr;60(4):317-326. doi: 10.1136/jmg-2022-109104. Epub 2023 Feb 27. PMID: 36849229.
Lung immune signatures define two groups of end-stage IPF patients
Authors: Cruz T, Mendoza N, Casas-Recasens S, Noell G, Hernandez-Gonzalez F, Frino-Garcia A, Alsina-Restoy X,
Molina M, Rojas M, Agustí A, Sellares J, Faner R. Respir Res. 2023 Sep 28;24(1):236. doi: 10.1186/s12931-023-02546-8. PMID: 37770891
Benefits of a Homecare Integral Patient Support Program in Idiopathic Pulmonary Fibrosis
Authors: Cobo Sanchez L, Ruiz Herrero C, Cobos I, Bespin J, Gadea C, Cerdá-Cortés P, Palma J, Vicens-Zygmunt V, Bermudo G, Portillo K, Boldova A, Robles A, Arribas J, Figuerola JA, Blavia R, Moreno A, Castillo D, Villar A, Belmonte Y, Badenes-Bonet D, Zayas D, Suarez-Cuartin G, Garcia-Bellmunt L, Sellarés J, Barril S, Sans J, Serra M, Perich D, Esteban LL, Esteban L, Balcells E, Gil F, Ruiz E, Fina C, Molina-Molina M. . Arch Bronconeumol. 2023 Aug;59(8):526-530. doi: 10.1016/j.arbres.2023.03.008. Epub 2023 Mar 21. PMID: 37003881
Functional Criteria to Define Progressive Pulmonary Fibrosis: Searching for the Holy Grail.
Authors: Noboa-Sevilla M, Hernández-González F, Alsina-Restoy X, Pérez-Rodas N, Sellarés J. Am J Respir Crit Care Med. 2023 Feb 1;207(3):368-369. doi: 10.1164/rccm.202209-1772LE. PMID: 36174219
Blood Immunophenotypes of Idiopathic Pulmonary Fibrosis: Relationship with Disease Severity and Progression.
Authors: Mendoza N, Casas-Recasens S, Olvera N, Hernandez-Gonzalez F, Cruz T, Albacar N, Alsina-Restoy X, Frino-Garcia A, López-Saiz G, Robres L, Rojas M, Agustí A, Sellarés J, Faner R. Int J Mol Sci. 2023 Sep 7;24(18):13832. doi: 10.3390/ijms241813832. PMID: 37762135
Eosinophils-from cradle to grave.
Authors: 1/JESENAK, Milos* – DIAMANT, Zuzana* (1.LF/870) – SIMON, Dagmar – TUFVESSON, Ellen – SEYS, Sven F. – MUKHERJEE, Manali – LACY, Paige – VIJVERBERG, Susanne – SLISZ, Tomáš (1.LF/870) – ŠEDIVÁ, Anna (2.LF/Ústav imunologie, FNM/9972) – SIMON, Hans-Uwe – STRIZ, Ilja – PLEVKOVA, Jana – SCHWARZE, Jurgen – KOSTURIAK, Radovan – ALEXIS, Neil E. – UNTERSMAYR, Eva – KOZIAR VAŠÁKOVÁ, Martina (1.LF/870) – KNOL, Edward – KOENDERMAN, Leo In: Allergy, 2023, 78(12):3077-3102, 26 s. ISSN 0105-4538
Pathologic Criteria for the Diagnosis of Usual Interstitial Pneumonia vs Fibrotic Hypersensitivity Pneumonitis in Transbronchial Cryobiopsies.
Authors: CHURG, Andrew* – TAZELAAR, Henry – MATĚJ, Radoslav (1.LF/310, 3.LF/Patmol, VFN/N854) – KOZIAR VAŠÁKOVÁ, Martina (1.LF/870) – STEWART, Brian – PATEL, Divya – DUARTE, Ernesto – GOMEZ MANJARRES, Diana C. – HIREN, Mehta J. – WRIGHT, Joanne L. In: Modern Pathology, 2023, 36(9):100221, 8 s. ISSN 0893-3952
The effect of nintedanib on lung functions and survival in idiopathic pulmonary fibrosis: real-life analysis of the Czech EMPIRE registry.
Authors: ŠTEFÁNIKOVÁ, Marianna* – DOUBKOVÁ, Martina – OVESNÁ, Petra – ŠTERCLOVÁ, Martina (1.LF/870, 2.LF/Pneumologická klinika) – LACINA, Ladislav – ŽURKOVÁ, Monika – PLAČKOVÁ, Martina – BARTOŠ, Vladimír (FNHK/PLICNÍ) – JANÍČKOVÁ, Ivana – BITTENGLOVÁ, Radka (FNP/KPF, LFPl/KPF) – ANTON, Jan (1.LF/870) – SÝKOROVÁ, Ľubica – LOŠŤÁKOVÁ, Vladimíra – MUSILOVÁ, Pavlína – ŠULDOVÁ, Hana – MOKOŠOVÁ, Radka – DIDYK, Jurij – ŠIŠÁKOVÁ, Lenka – LISÁ, Pavlína (2.LF/Pneumologická klinika, FNM/2115) – LNĚNIČKA, Jaroslav – DAŘIČKOVÁ, Hana – DOLEŽEL, Daniel – PŠIKALOVÁ, Jana – TYL, Richard – KRÁLOVÁ, Renata – KOZIAR VAŠÁKOVÁ, Martina (1.LF/870) In: BMC Pulmonary Medicine, 2023, 23(1):154, 9 s. ISSN 1471-2466
Reference českých pacientů s idiopatickou plicní fibrózou do transplantačního centra – data z registru EMPIRE.
Authors: ŠTERCLOVÁ, Martina* (1.LF/870, 2.LF/Pneumologická klinika) – DOUBKOVÁ, M. – SÝKOROVÁ, Ľ. – BARTOŠ, Vladimír (FNHK/PLICNÍ) – ŽURKOVÁ, M. – LOŠŤÁKOVÁ, V. – MOKOŠOVÁ, R. – PLAČKOVÁ, M. – LACINA, L. – CIMROVÁ, M. – BITTENGLOVÁ, Radka (FNP/KPF) – LISÁ, Pavlína (2.LF/Pneumologická klinika, FNM/2115) – MUSILOVÁ, P. – DOLEŽAL, D. – PŠIKALOVÁ, J. – OVESNÁ, P. – KOZIAR VAŠÁKOVÁ, Martina (1.LF/870) (AJ název: Referring Czech patients with idiopathic pulmonary fibrosis to a transplant center – data from the EMPIRE registry) In: Studia Pneumologica et Phthiseologica, 2023, 83(3):104-111, 8 s. ISSN 1213-810X
Pulmonary fibrosis: from pathogenesis to clinical decision-making.
Authors: Koudstaal T, Funke-Chambour M, Kreuter M, Molyneaux PL, Wijsenbeek MS. Trends Mol Med. 2023 Dec;29(12):1076-1087. doi: 10.1016/j.molmed.2023.08.010. Epub 2023 Sep 14. PMID: 37716906 Review.
Ziritaxestat and Lung Function in Idiopathic Pulmonary Fibrosis-Reply.
Authors: Maher TM, Ford P, Wijsenbeek MS. JAMA. 2023 Sep 12;330(10):973-974. doi: 10.1001/jama.2023.12640. PMID: 37698565 No abstract available.
Differentiating interstitial lung diseases from other respiratory diseases using electronic nose technology.
Authors: van der Sar IG, Wijsenbeek MS, Braunstahl GJ, Loekabino JO, Dingemans AC, In ‘t Veen JCCM, Moor CC. Respir Res. 2023 Nov 6;24(1):271. doi: 10.1186/s12931-023-02575-3. PMID: 37932795 Free PMC article.
Palliative care in COPD and ILD: a call for action.
Authors: Wijsenbeek M, Valenzuela C, Holland A. Eur Respir J. 2023 Aug 17;62(2):2301076. doi: 10.1183/13993003.01076-2023. Print 2023 Aug. PMID: 37591552 No abstract available.
I-PreFer Study: A Discrete Choice Experiment to Explore Patient, Caregiver and Pulmonologist Preferences of Idiopathic Pulmonary Fibrosis Pharmacological Treatment Options.
Authors: Hollmen M, Wijsenbeek M, Bromilow T, Smith AB, Mealing S, Lewis D, Galvin L, Jones S, Asijee G, Soulard S, Froidure A. Patient Prefer Adherence. 2023 Aug 4;17:1895-1906. doi: 10.2147/PPA.S409767. eCollection 2023. PMID: 37560147 Free PMC article.
Exhaled breath analysis in interstitial lung disease.
Authors: van der Sar IG, Wijsenbeek MS, Moor CC. Curr Opin Pulm Med. 2023 Sep 1;29(5):443-450. doi: 10.1097/MCP.0000000000000978. Epub 2023 Jul 5. PMID: 37405699 Free PMC article.
Review. The lung way home: ready for home monitoring in lung diseases?
Authors: Moor CC, Wijsenbeek MS. Curr Opin Pulm Med. 2023 Jul 1;29(4):256-258. doi: 10.1097/MCP.0000000000000970. PMID: 37254922 No abstract available.
Role of the internet of medical things in care for patients with interstitial lung disease.
Authors: Nakshbandi G, Moor CC, Wijsenbeek MS. Curr Opin Pulm Med. 2023 Jul 1;29(4):285-292. doi: 10.1097/MCP.0000000000000971. Epub 2023 May 19. PMID: 37212372 Free PMC article. Review.
Idiopathic pulmonary fibrosis.
Authors: Koudstaal T, Wijsenbeek MS. Presse Med. 2023 May 6;52(3):104166. doi: 10.1016/j.lpm.2023.104166. Online ahead of print. PMID: 37156412
Holistic management of patients with progressive pulmonary fibrosis.
Authors: Oliveira A, Fabbri G, Gille T, Bargagli E, Duchemann B, Evans R, Pinnock H, Holland AE, Renzoni E, Ekström M, Jones S, Wijsenbeek M, Dinh-Xuan AT, Vagheggini G. Breathe (Sheff). 2023 Sep;19(3):230101. doi: 10.1183/20734735.0101-2023. Epub 2023 Sep 12. PMID: 37719243 Free PMC article. Review.
Inhaled pirfenidone solution (AP01) for IPF: a randomised, open-label, dose-response trial. Authors: West A, Chaudhuri N, Barczyk A, Wilsher ML, Hopkins P, Glaspole I, Corte TJ, Šterclová M, Veale A, Jassem E, Wijsenbeek MS, Grainge C, Piotrowski W, Raghu G, Shaffer ML, Nair D, Freeman L, Otto K, Montgomery AB. Thorax. 2023 Sep;78(9):882-889. doi: 10.1136/thorax-2022-219391. Epub 2023 Mar 22. PMID: 36948586 Clinical Trial.
The flare of care for rare: per aspera ad astra for rare lung diseases!
Authors: Wijsenbeek M, Humbert M, Wagner T, Kreuter M. Eur Respir Rev. 2023 Feb 7;32(167):230006. doi: 10.1183/16000617.0006-2023. Print 2023 Mar 31. PMID: 36754435 Free PMC article.
The world of rare interstitial lung diseases.
Authors: Buschulte K, Cottin V, Wijsenbeek M, Kreuter M, Diesler R. Eur Respir Rev. 2023 Feb 7;32(167):220161. doi: 10.1183/16000617.0161-2022. Print 2023 Mar 31. PMID: 36754433 Free PMC article. Review.
Study protocol of an international patient-led registry in patients with pulmonary fibrosis using online home monitoring: I-FILE.
Authors: Nakshbandi G, Moor CC, Antoniou K, Cottin V, Hoffmann-Vold AM, Koemans EA, Kreuter M, Molyneaux PL, Wuyts WA, Wijsenbeek MS. BMC Pulm Med. 2023 Feb 2;23(1):51. doi: 10.1186/s12890-023-02336-4. PMID: 36732734 Free PMC article.
Home monitoring in interstitial lung diseases.
Authors: Wijsenbeek MS, Moor CC, Johannson KA, Jackson PD, Khor YH, Kondoh Y, Rajan SK, Tabaj GC, Varela BE, van der Wal P, van Zyl-Smit RN, Kreuter M, Maher TM. Lancet Respir Med. 2023 Jan;11(1):97-110. doi: 10.1016/S2213-2600(22)00228-4. Epub 2022 Oct 4. PMID: 36206780 Review.
Risk Stratifying Interstitial Lung Abnormalities to Guide Early Diagnosis of Interstitial Lung Diseases.
Authors: Wijsenbeek MS, Brusselle GG. Am J Respir Crit Care Med. 2023 Jan 1;207(1):9-11. doi: 10.1164/rccm.202209-1817ED. PMID: 36170647 Free PMC article. No abstract available.
Leukocyte telomere length: the dawn of a new era of personalised medicine in fibrotic interstitial lung diseases?
Authors: Cottin V, Kolb M. Eur Respir J. 2023 Nov 29;62(5):2301852. doi: 10.1183/13993003.01852-2023. PMID: 38035695.
Outcome of lung transplantation for adults with interstitial lung disease associated with genetic disorders of the surfactant system.
Authors: Bermudez J, Nathan N, Coiffard B, Roux A, Hirschi S, Degot T, Bunel V, Le Pavec J, Macey J, Le Borgne A, Legendre M, Cottin V, Thomas PA, Borie R, Reynaud-Gaubert M. ERJ Open Res. 2023 Nov 20;9(6):00240-2023. doi: 10.1183/23120541.00240-2023. PMID: 38020562; PMCID: PMC10658627.
Idiopathic pulmonary fibrosis with benign SFTPC variant and pathogenic MARS1 mutations: can’t see the forest for the trees!
Authors: Castaldo A, Delestrain C, Diesler R, Merveilleux du Vignaux C, Onnee M, Touraine R, Chalabreysse L, Fanen P, Epaud R, Cottin V, De Becdelièvre A. ERJ Open Res. 2023 Nov 6;9(6):00472-2023. doi: 10.1183/23120541.00472-2023. PMID: 37936900; PMCID: PMC10626413.
Infections in autoimmune pulmonary alveolar proteinosis: a large retrospective cohort.
Authors: Mabo A, Borie R, Wemeau-Stervinou L, Uzunhan Y, Gomez E, Prevot G, Reynaud- Gaubert M, Traclet J, Bergot E, Cadranel J, Marchand-Adam S, Bergeron A, Blanchard E, Bondue B, Bonniaud P, Bourdin A, Burgel PR, Hirschi S, Marquette CH, Quétant S, Nunes H, Chenivesse C, Crestani B, Guirriec Y, Monnier D, Ménard C, Tattevin P, Cottin V, Luque Paz D, Jouneau S; OrphaLung Network. Thorax. 2023 Dec 15;79(1):68-74. doi: 10.1136/thorax-2023-220040. PMID: 37758458.
Design of a phase III, double-blind, randomised, placebo-controlled trial of BI 1015550 in patients with progressive pulmonary fibrosis (FIBRONEER-ILD).
Authors: Maher TM, Assassi S, Azuma A, Cottin V, Hoffmann-Vold AM, Kreuter M, Oldham JM, Richeldi L, Valenzuela C, Wijsenbeek MS, Coeck C, Schlecker C, Voss F, Wachtlin D, Martinez FJ. BMJ Open Respir Res. 2023 Sep;10(1):e001580. doi: 10.1136/bmjresp-2022-001580. PMID: 37709661; PMCID: PMC10503394.
Alpha1-antitrypsin deficiency: An updated review.
Authors: Mornex JF, Traclet J, Guillaud O, Dechomet M, Lombard C, Ruiz M, Revel D, Reix P, Cottin V. Presse Med. 2023 Jul 29;52(3):104170. doi: 10.1016/j.lpm.2023.104170. Epub ahead of print. PMID: 37517655.
Interstitial lung disease- associated pulmonary hypertension – what the future holds.
Authors: Cottin V, Diesler R, Turquier S, Valenzuela C. Curr Opin Pulm Med. 2023 Sep 1;29(5):406-415. doi: 10.1097/MCP.0000000000000992. Epub 2023 Jul 7. PMID: 37417835.
Inhaled treprostinil for interstitial lung disease-associated pulmonary hypertension: a silver lining on a very dark cloud.
Authors: Cottin V, Valenzuela C, Humbert M. Eur Respir J. 2023 Jun 29;61(6):2300944. doi: 10.1183/13993003.00944-2023. PMID: 37385654.
Treatable traits: a comprehensive precision medicine approach in interstitial lung disease.
Authors: Khor YH, Cottin V, Holland AE, Inoue Y, McDonald VM, Oldham J, Renzoni EA, Russell AM, Strek ME, Ryerson CJ. Eur Respir J. 2023 Jul 27;62(1):2300404. doi: 10.1183/13993003.00404-2023. PMID: 37263752; PMCID: PMC10626565.
Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis withpolyangiitis.
Authors: Emmi G, Bettiol A, Gelain E, Bajema IM, Berti A, Burns S, Cid MC, Cohen Tervaert JW, Cottin V, Durante E, Holle JU, Mahr AD, Del Pero MM, Marvisi C, Mills J, Moiseev S, Moosig F, Mukhtyar C, Neumann T, Olivotto I, Salvarani C, Seeliger B, Sinico RA, Taillé C, Terrier B, Venhoff N, Bertsias G, Guillevin L, Jayne DRW, Vaglio A. Nat Rev Rheumatol. 2023 Jun;19(6):378-393. doi: 10.1038/s41584-023-00958-w. Epub 2023 May 9. PMID: 37161084.
French recommendations for the diagnosis and management of lymphangioleiomyomatosis.
Authors: Cottin V, Blanchard E, Kerjouan M, Lazor R, Reynaud-Gaubert M, Taille C, Uzunhan Y, Wemeau L, Andrejak C, Baud D, Bonniaud P, Brillet PY, Calender A, Chalabreysse L, Court-Fortune I, Desbaillets NP, Ferretti G, Guillemot A, Hardelin L, Kambouchner M, Leclerc V, Lederlin M, Malinge MC, Mancel A, Marchand-Adam S, Maury JM, Naccache JM, Nasser M, Nunes H, Pagnoux G, Prévot G, Rousset-Jablonski C, Rouviere O, Si-Mohamed S, Touraine R, Traclet J, Turquier S, Vagnarelli S, Ahmad K; OrphaLung network. Respir Med Res. 2023 Jun;83:101010. doi:10.1016/j.resmer.2023.101010. Epub 2023 Mar 24. PMID: 37087906.
Lung Dual-Energy CT Perfusion Blood Volume as a Marker of Severity in Chronic Thromboembolic Pulmonary Hypertension.
Authors: Si-Mohamed SA, Zumbihl L, Turquier S, Boccalini S, Mornex JF, Douek P, Cottin V, Boussel L. Diagnostics (Basel). 2023 Feb 17;13(4):769. doi:10.3390/diagnostics13040769. PMID: 36832256; PMCID: PMC9955200.
The world of rare interstitial lung diseases.
Authors: Buschulte K, Cottin V, Wijsenbeek M, Kreuter M, Diesler R. Eur Respir Rev. 2023 Feb 7;32(167):220161. doi: 10.1183/16000617.0161-2022. PMID: 36754433; PMCID: PMC9910344.
French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis – 2021 update. Full-length version.
Authors: Cottin V, Bonniaud P, Cadranel J, Crestani B, Jouneau S, Marchand-Adam S, Nunes H, Wémeau-Stervinou L, Bergot E, Blanchard E, Borie R, Bourdin A, Chenivesse C, Clément A, Gomez E, Gondouin A, Hirschi S, Lebargy F, Marquette CH, Montani D, Prévot G, Quetant S, Reynaud-Gaubert M, Salaun M, Sanchez O, Trumbic B, Berkani K, Brillet PY, Campana M, Chalabreysse L, Chatté G, Debieuvre D, Ferretti G, Fourrier JM, Just N, Kambouchner M, Legrand B, Le Guillou F, Lhuillier JP, Mehdaoui A, Naccache JM, Paganon C, Rémy-Jardin M, Si-Mohamed S, Terrioux P; OrphaLung network. Respir Med Res. 2023 Jun;83:100948. doi: 10.1016/j.resmer.2022.100948. Epub 2022 Aug 4. PMID: 36630775.
Progressive pulmonary fibrosis: an expert group consensus statement.
Authors: Rajan SK, Cottin V, Dhar R, Danoff S, Flaherty KR, Brown KK, Mohan A, Renzoni E, Mohan M, Udwadia Z, Shenoy P, Currow D, Devraj A, Jankharia B, Kulshrestha R, Jones S, Ravaglia C, Quadrelli S, Iyer R, Dhooria S, Kolb M, Wells AU. Eur Respir J. 2023 Mar 30;61(3):2103187. doi: 10.1183/13993003.03187-2021. PMID: 36517177; PMCID: PMC10060665.
GLPG1205 for idiopathic pulmonary fibrosis: a phase 2 randomised placebo-controlled trial.
Authors: Strambu IR, Seemayer CA, Fagard LMA, Ford PA, Van der Aa TAK, de Haas- Amatsaleh AA, Modgill V, Santermans E, Sondag EN, Helmer EG, Maher TM, Costabel U, Cottin V. Eur Respir J. 2023 Mar 2;61(3):2201794. doi: 10.1183/13993003.01794-2022. PMID: 36328358; PMCID: PMC9978158.
Clinical course of suspected familial and sporadic idiopathic pulmonary fibrosis: Data from the PROOF-Next registry.
Authors: Froidure A, Bondue B, Dahlqvist C, Guiot J, Gusbin N, Wirtz G, Brusselle G, Strens D, Slabbynck H, Wuyts WA.
Respirology. 2023 Dec 20. doi: 10.1111/resp.14650.
Ultra-rare RTEL1 gene variants associate with acute severity of COVID-19 and evolution to pulmonary fibrosis as a specific long COVID disorder.
Authors: Laura Bergantini , Margherita Baldassarri , Miriana d’Alessandro, Giulia Brunelli, Gaia Fabbri, Kristina Zguro, Andrea Degl’Innocenti; GEN-COVID Multicenter study; Chiara Fallerini, Elena Bargagli, Alessandra Renieri. Respir Res. 2023 Jun 16;24(1):158. doi: 10.1186/s12931-023-02458-7.
Interstitial lung disease with and without progressive fibrosing phenotype in patients with idiopathic inflammatory myopathies: data from a large multicentric cohort.
Authors: Zanatta E, Cocconcelli E, Castelli G, Giraudo C, Fraia AS, De Zorzi E, Gatto M, Ienna L, Treppo E, Malandrino D, Cereser L, Emmi G, Giannelli F, Bellani S, Martini A, Moccaldi B, Ghirardello A, Avouac J, Quartuccio L, Allanore Y, Doria A, Spagnolo P, Balestro E, Iaccarino L. RMD Open. 2023 Aug;9(3):e003121.
Environmental Causes of Idiopathic Pulmonary Fibrosis.
Authors: Sheiphali Gandhi S, Tonelli R, Murray M, Samarelli AV, Spagnolo P. Int J Mol Sci. 2023. Manuscript ID: ijms-2657096
Computed Tomographic Phenotypes in Pulmonary Sarcoidosis – Results of a Multinational Delphi Study.
Authors: Desai SR, Sivarasan N, Johannson KA, George PM, Culver DA, Devaraj A, Lynch DA, Milne D, Renzoni E, Nunes H, Sverzellati N, Spagnolo P, Baughman R, Yadav R, Piciucchi S, Walsh SLF, Kouranos V, Wells AU. Lancet Respir Med 2023. LANRES2829.
ERS international congress 2023: highlights from the Interstitial Lung Diseases Assembly
Authors: Fabbri L, Guiot J, M Vermant, Miadlikowska E, Estrella D, Wijsenbeek M, Wuyts W, Bargagli E, Froidure A, Spagnolo P, Veltkamp M, Molina-Molina M, McCarthy C. Antoniou K, Kreuter M, Moor C. ERJ Open Research (ERJOR-00839-2023).
An adjudication algorithm for respiratory-related hospitalisation in IPF.
Authors: Ford P, Kreuter M, Brown KK, Wuyts WA, Wijsenbeek M, Israël-Biet D, Hubbard R, Nathan SD, Nunes H, Penninckx B, Prasad N, Seghers I, Spagnolo P, Verbruggen N, Hirani N, Behr J, Kaner RJ, Maher TM. ERJ Open Research ERJOR-00636-2023.R1.
Emerging treatments for childhood interstitial lung disease.
Authors: Bernardinello N, Griese M, Borie R, Spagnolo P. Paediatr Drugs. 2023 Nov 10.
8. Bonella F, Spagnolo P, Ryerson C. Current and Future Treatment Landscape for Idiopathic Pulmonary Fibrosis. Drugs. 2023 Oct 26.
Should we use nintedanib as early therapy in patients with SSc-ILD?
Authors: Zanatta E, Moccaldi B, Szucs G, Spagnolo P. Autoimmun Rev. 2023 Oct 14:103463.
10. Spagnolo P, Ryerson CJ, Guler S, Feary J, Churg A, Fontenot AP, Piciucchi S, Udwadia Z, Corte TJ, Wuyts WA, Johannson KA, Cottin V. Occupational interstitial lung diseases. J Intern Med. 2023 Aug 3.
Healthcare resource use and associated costs in patients receiving pirfenidone or nintedanib for idiopathic pulmonary fibrosis.
Authors: Cottin V, Spagnolo P, Bonniaud P, Dalon F, Nolin M, Kirchgässler KU, Van Ganse E, Belhassen M. Respir Med Res. 2023 Jun;83:100951.
Significant functional improvement in IPF patient treated with antifibrotic drugs: preliminary results and clinical outcome.
Authors: Caminati A, Awad I, Elia D, Cassandro R, Mozzanica F, Pelosi G, Zompatori M, Harari S. Minerva Med. 2023 Oct;114(5):736-738. doi: 10.23736/S0026-4806.23.08720-7.
Comparison of clinical scoring to predict mortality risk in mild-to-moderate idiopathic pulmonary fibrosis.
Authors: Sonaglioni A, Caminati A, Elia D, Trevisan R, Zompatori M, Grasso E, Lombardo M, Harari S. Minerva Med. 2023 Oct;114(5):608-619. doi: 10.23736/S0026-4806.23.08585-3.
Coronary artery calcium score is a prognostic factor for mortality in idiopathic pulmonary fibrosis.
Authors: Caminati A. Zompatori M, Fuccillo N, Sonaglioni A, Elia D, Cassandro R, Trevisan R, Pelosi G, Harari S. Minerva Medica 2023; 114(6): 815-824. doi: 10.23736/S0026-4806.22.080181.
Lymphangioleiomyomatosis.
Authors: Elia D, Cassandro R, Caminati A, Luisi F, Harari S. Presse Med. 2023 Sep 9;52(3):104173. doi: 10.1016/j.lpm.2023.104173. Online ahead of print.
Short-Term Evolution of Nutritional Status in Patients with Idiopathic Pulmonary Fibrosis.
Authors: Faverio P, Fumagalli A, Conti S, Madotto F, Bini F, Harari S, Mondoni M, Oggionni T, Barisione E, Ceruti P, Papetti MC, Bodini BD, Caminati A, Valentino A, Centanni S, Lanzi P, Della Zoppa M, Crotti S, Grosso M, Sukkar SG, Modina D, Andreoli M, Nicali R, Suigo G, Busnelli S, Paciocco G, Lettieri S, Mantovani LG, Cesana G, Pesci A, Luppi F. Ann Am Thorac Soc. 2023 Jul;20(7):1066-1070. doi: 10.1513/AnnalsATS.202211-935RL.
Prognostic role of CHA2DS2-VASc score for mortality risk assessment in non-advanced idiopathic pulmonary fibrosis: a preliminary observation.
Authors: Sonaglioni A, Caminati A, Re M, Elia D, Trevisan R, Granato A, Zompatori M, Lombardo M, Harari S. Intern Emerg Med. 2023 Apr;18(3):755-767. doi: 10.1007/s11739-023-03219-6
Now we know: chronic exposure to air pollutants is a risk factor for the development of idiopathic pulmonary fibrosis.
Authors: Sesé L, Harari S. Eur Respir J. 2023 Feb 2;61(2):2202113. doi: 10.1183/13993003.02113-2022.
Editorial: Idiopathic pulmonary fibrosis: epidemiology, prognosis and treatment.
Authors: Caminati A, Meloni F, Fujita M. Front Med (Lausanne). 2023 Jun 1;10:1195263. doi: 10.3389/fmed.2023.1195263. eCollection 2023.
Clinical course of suspected familial and sporadic idiopathic pulmonary fibrosis: Data from the PROOF-Next registry. Authors: Froidure A, Bondue B, Dahlqvist C, Guiot J, Gusbin N, Wirtz G, Brusselle G, Strens D, Slabbynck H, Wuyts WA. Respirology. 2023 Dec 20.
Treatment and Prognosis of Patients with Lung Cancer and Combined Interstitial Lung Disease.
Authors: Mauclet C, Dupont MV, Roelandt K, Regnier M, Delos M, Pirard L, Vander Borght T, Dahlqvist C, Froidure A, Rondelet B, Vanderick J, Remouchamps V, Duplaquet F, Ocak S. Cancers (Basel). 2023 Jul 30;15(15):3876
I-PreFer Study: A Questionnaire to Explore Patient, Caregiver and Pulmonologist Preferences of Idiopathic Pulmonary Fibrosis Treatment Options. Authors: Hollmen M, Bromilow T, Smith AB, Mealing S, Lewis D, Galvin L, Jones S, Pacheco L, Soulard S, Froidure A. Patient Prefer Adherence. 2023 Aug 4;17:1621-1639
Identification of Paired-related Homeobox Protein 1 as a key mesenchymal transcription factor in pulmonary fibrosis. Authors: Marchal-Duval E, Homps-Legrand M, Froidure A, Jaillet M, Ghanem M, Lou D, Justet A, Maurac A, Vadel A, Fortas E, Cazes A, Joannes A, Giersh L, Mal H, Mordant P, Piolot T, Truchin M, Mounier CM, Schirduan K, Korfei M, Gunther A, Mari B, Jaschinski F, Crestani B, Mailleux AA. Elife. 2023 Jun 1;12:e79840
Correlation of BAL Cell Count and Pulmonary Function Tests in the Era of Antifibrotics: Data From the Belgium-Luxembourg Idiopathic Pulmonary Fibrosis Registry.
Authors: Froidure A, Bondue B, Dahlqvist C, Guiot J, Gusbin N, Wirtz G, Joos G, Cataldo D, Strens D, Slabbynck H, Wuyts WA. Chest. 2023 Feb;163(2):358-361
2022
[French practical guidelines for the diagnosis and management of IPF – 2021 update, short version].
Authors: Cottin V, Bonniaud P, Cadranel J, Crestani B, Jouneau S, Marchand-Adam S, Nunes H, Wémeau-Stervinou L, Bergot E, Blanchard E, Borie R, Bourdin A, Chenivesse C, Clément A, Gomez E, Gondouin A, Hirschi S, Lebargy F, Marquette CH, Montani D, Prévot G, Quetant S, Reynaud-Gaubert M, Salaun M, Sanchez O, Trumbic B, Berkani K, Brillet PY, Campana M, Chalabreysse L, Chatté G, Debieuvre D, Ferretti G, Fourrier JM, Just N, Kambouchner M, Legrand B, Le Guillou F, Lhuillier JP, Mehdaoui A, Naccache JM, Paganon C, Rémy-Jardin M, Si-Mohamed S, Terrioux P; OrphaLung.Rev Mal Respir. 2022 Mar;39(3):275-312. doi: 10.1016/j.rmr.2022.01.005. Epub 2022 Mar 15.PMID: 35304014
Authors: Verónica Pulito Cueto; Sara Remuzgo Martínez; Fernanda Genre; Belén Atienza Mateo; Víctor Manuel Mora Cuesta; David Iturbe Fernández; Leticia Lera Gómez; Javier Rodríguez Carrio; Diana Prieto Peña; VIrginia Portilla González; Ricardo Blanco Alonso; Alfondo Fernando Corrales Martínez; Oreste Gualillo; José Manuel Cifrián Martínez; Raquel López Mejías; Miguel Ángel González Gay. Biomedicines. MDPI, 05/04/2022. DOI: 10.3390/biomedicines10040851
Authors: María Iglesias Escudero; David San Segundo Arribas; David Merino Fernández; Víctor Manuel Mora Cuesta; Patricia Lamadrid Perojo; Marta Alonso Peña; Sandra Raso Torres; David Iturbe Fernández; Sonia Fernández Rozas; José Manuel Cifrián Martínez; Marcos López Hoyos. Frontiers in Inmunology. 10/01/2022. DOI: 10.3389/fimmu.2021.788851
Authors: Víctor Manuel Mora Cuesta; María de los Ángeles Ballesteros Sanz; Sara Naranjo Gozalo; Laura Sánchez Moreno; Borja Suberviola Cañas; David Iturbe Fernández; Bonifacio Cimadevilla Calvo; Sandra Tello Mena; Carlos Álvarez de Arriba; Eduardo Miñambres García. American Journal of Transplantation. Wiley, 07/04/2022. DOI: 10.1111/ajt.17057
Authors: Rita Nogueiras Álvarez; Víctor Manuel Mora Cuesta; José Manuel Cifrián Martínez; María de los Ángeles Cos Cossío; María del Mar García Saiz Nature Publishing Group, 12/12/2022. DOI: 10.1038/s41598-022-25445-2
Authors: Marta Cristeto Porras; Víctor Manuel Mora Cuesta; David Iturbe Fernández; Sandra Tello Mena; Pilar Alonso Lecue; Laura Sánchez Moreno; Eduardo Miñambres García; Sara Naranjo Gozalo; Sheila Izquierdo Cuervo; José Manuel Cifrián Martínez. Wiley, 26/10/2022. DOI: 10.1111/ctr.14832
Authors: Víctor Manuel Mora Cuesta; Amaya Martínez Meñaca; David Iturbe Fernández; Sandra Tello Mena; Pilar Alonso Lecue; Daniel Fernández Márquez; Begoña Sáinz Ezquerra Belmonte; María José Gallardo Ruiz; José Manuel Cifrián Martínez. Wiley, 30/10/2022. DOI: 10.1002/pul2.12149
Evaluation of Two Different CMV-Immunoglobulin Regimens for Combined CMV Prophylaxis in High-Risk Patients following Lung Transplant.
Authors: Víctor Manuel Mora Cuesta; Piedad Usstti Gil; Alicia De Pablo Gafas; David Iturbe Fernández; Rosalía Laporta Hernández; Rodrigo Alonso Moralejo; Myriam Aguilar Pérez; Carlos Andrés Quezada Loaiza; José Manuel Cifrián Martínez. Microorganisms. MDPI, 22/12/2022. DOI: 10.3390/microorganisms11010032
European Respiratory Society International Congress 2021: highlights from best-abstract awardees.
Authors: Sanjay Ramakrishnan, Fabien Beaufils, Jana De Brandt, Kerri Viney, Claire Bradley, Vincent Cottin, Maged Hassan, Joana Cruz. Breathe (Sheff). 2022 Mar;18(1):210176.doi: 10.1183/20734735.0176-2021. Epub 2022 Apr 5.
Authors: Si-Mohamed SA, Cierco A, Gamondes D, Restier LM, Delagrange L, Cottin V, Dupuis-Girod S, Revel D.J Pers Med. 2022 Jun 30;12(7):1091. doi: 10.3390/jpm12071091.PMID: 35887588 Free PMC article.
Clinical impact of TERT somaticmutation in telomerase-related gene mutation carriers after lung transplantation.
Authors: Ibrahima Ba, Caroline Kannengiesser, Hervé Mal, Martine Reynaud-Gaubert, Vincent Cottin, Sandrine Hirschi, Clément Picard, Raphael Borie. J Heart Lung Transplant. 2022 Sep;41(9):1207-1209. doi: 10.1016/j.healun.2022.06.012. Epub 2022 Jun 19.
GLPG1205 for idiopathic pulmonary fibrosis: a Phase 2 randomised placebo-controlled trial.
Authors: Strambu IR, Seemayer CA, Fagard LMA, Ford PA, Van der Aa TAK, de Haas-Amatsaleh AA, Modgill V, Santermans E, Sondag EN, Helmer EG, Maher TM, Costabel U, Cottin V.Eur Respir J. 2022 Nov 3:2201794. doi: 10.1183/13993003.01794-2022. Online ahead of print.PMID: 36328358 Free article.
Authors: Daniël A. Korevaar, Sara Colella, Markus Fally, Juliette Camuset, Thomas V. Colby, Lars Hagmeyer, Juergen Hetzel, Fabien Maldonado, Antonio Morais, Claudia Ravaglia, René Spijker, Sara Tomassetti, Lauren K. Troy, Johny A. Verschakelen, Athol U. Wells, Thomy Tonia, Jouke T. Annema, Venerino Poletti. European Respiratory Journal 2022 60: 2200425; DOI: 10.1183/13993003.00425-2022
Respiratory management of spinal muscular atrophy type 1 patients treated with Nusinersen.
Authors: Joris Menard, Andreea M Seferian, Emmanuelle Fleurence, Audrey Barzic, Alexandra Binoche, Géraldine Labouret, Laurianne Coutier, Carole Vuillerot, Blaise M Bieleu, Marta Gomez Garcia de la Banda, Harriet Corvol, Laurent Servais, Jessica Taytard. Pediatr Pulmonol. 2022 Jun;57(6):1505-1512. doi: 10.1002/ppul.25899. Epub 2022 Mar 28.
Authors: Maria Molina-Molina, Michael Kreuter, Vincent Cottin, Tamera J Corte, Frank Gilberg, Klaus-Uwe Kirchgaessler, Judit Axmann, Toby M Maher. Front Med (Lausanne). 2022 Jun 17;9:897102. doi: 10.3389/fmed.2022.897102. eCollection 2022.
Authors: Vincent Cottin, Sara Tomassetti, Claudia Valenzuela, Simon L F Walsh, Katerina M Antoniou, Francesco Bonella, Kevin K Brown, Harold R Collard, Tamera J Corte, Kevin R Flaherty, Kerri A Johannson, Martin Kolb, Michael Kreuter, Yoshikazu Inoue, R Gisli Jenkins, Joyce S Lee, David A Lynch, Toby M Maher, Fernando J Martinez, Maria Molina-Molina, Jeff L Myers, Steven D Nathan, Venerino Poletti, Silvia Quadrelli, Ganesh Raghu, Sujeet K Rajan, Claudia Ravaglia, Martine Remy-Jardin, Elisabetta Renzoni, Luca K Richeldi, Paolo Spagnolo, Lauren Troy, Marlies Wijsenbeek, Kevin C Wilson, Wim Wuyts, Athol U Wells, Christopher J Ryerson
Authors: Jouneau S, Crestani B, Thibault R, Lederlin M, Vernhet L, Yang M, Morgenthien E, Kirchgaessler KU, Cottin V.Respiration. 2022;101(2):142-154. doi: 10.1159/000518855. Epub 2021 Oct 5.PMID: 34610600
Authors: Cottin V, Martinez FJ, Jenkins RG, Belperio JA, Kitamura H, Molina-Molina M, Tschoepe I, Coeck C, Lievens D, Costabel U.Respir Res. 2022 Apr 7;23(1):85. doi: 10.1186/s12931-022-01974-2.PMID: 35392908
Authors: Phillips-Houlbracq M, Mal H, Cottin V, Gauvain C, Beier F, Sicre de Fontbrune F, Sidali S, Mornex JF, Hirschi S, Roux A, Weisenburger G, Roussel A, Wémeau-Stervinou L, Le Pavec J, Pison C, Marchand Adam S, Froidure A, Lazor R, Naccache JM, Jouneau S, Nunes H, Reynaud-Gaubert M, Le Borgne A, Boutboul D, Ba I, Boileau C, Crestani B, Kannengiesser C, Borie R; OrphaLung Network.Am J Transplant. 2022 Apr;22(4):1236-1244. doi: 10.1111/ajt.16893. Epub 2021 Dec 28.PMID: 34854205
Authors: Kreuter M, Maher TM, Corte TJ, Molina-Molina M, Axmann J, Gilberg F, Kirchgaessler KU, Cottin V.Adv Ther. 2022 Feb;39(2):1081-1095. doi: 10.1007/s12325-021-02009-w. Epub 2021 Dec 22.PMID: 34936057
Authors: Grignaschi S, Mongodi S, Alfonsi E, Mojoli F, Vertui V, Zanframundo G, Cavagna L.Clin Exp Rheumatol. 2022 May;40 Suppl 134(5):121-123. doi: 10.55563/clinexprheumatol/0u7vdc. Epub 2022 Feb 10.PMID: 35238770
Authors: Philippot Q, Kannengiesser C, Debray MP, Gauvain C, Ba I, Vieri M, Gondouin A, Naccache JM, Reynaud-Gaubert M, Uzunhan Y, Bondue B, Israël-Biet D, Dieudé P, Fourrage C, Lainey E, Manali E, Papiris S, Wemeau L, Hirschi S, Mal H, Nunes H, Schlemmer F, Blanchard E, Beier F, Cottin V, Crestani B, Borie R; OrphaLung Network.Respirology. 2022 Mar;27(3):226-235. doi: 10.1111/resp.14195. Epub 2022 Jan 3.PMID: 34981600
Sjögren disease, not Sjögren’s: comment on the article by Baer and Hammitt.
Authors: Cottin V.Arthritis Rheumatol. 2022 Feb;74(2):366-367. doi: 10.1002/art.41952. Epub 2021 Dec 14.PMID: 3442392
Targeted therapy for pulmonary alveolar proteinosis: the time is now.
Authors: Bonella F, Borie R.Eur Respir J. 2022 Apr 21;59(4):2102971. doi: 10.1183/13993003.02971-2021. Print 2022 Apr.PMID: 35450922. Bonella F, Borie R.Eur Respir J. 2022 Apr 21;59(4):2102971. doi: 10.1183/13993003.02971-2021. Print 2022 Apr.PMID: 35450922
A call for evidence in connective tissue diseases-associated interstitial lung disease.
Authors: Cottin V, Lega JC, Coury F, Nasser M.Joint Bone Spine. 2022 Jan;89(1):105274. doi: 10.1016/j.jbspin.2021.105274. Epub 2021 Sep 15
The Burden of Progressive-Fibrosing Interstitial Lung Diseases.
Authors: Cottin V, Teague R, Nicholson L, Langham S, Baldwin M.Front Med (Lausanne). 2022 Feb 1;9:799912. doi: 10.3389/fmed.2022.799912. eCollection 2022.PMID: 35178411
Tobramycin safety and efficacy review article
Abstract: Carsten Schwarz, Giovanni Taccetti, Pierre-Regis Burgel, Siobhain Mulrennan. Respir Med. Apr-May 2022;195:106778. doi: 10.1016/j.rmed.2022.106778. Epub 2022 Feb 25.
Drug-induced interstitial lung disease.
Authors: Paolo Spagnolo, Philippe Bonniaud, Giulio Rossi, Nicola Sverzellati, Vincent Cottin. Eur Respir J. 2022 Mar 24;2102776. doi: 10.1183/13993003.02776-2021. Online ahead of print.
Trial of a Preferential Phosphodiesterase 4B Inhibitor for Idiopathic Pulmonary Fibrosis.
Authors: Luca Richeldi, Arata Azuma, Vincent Cottin, Christian Hesslinger, Susanne Stowasser, Claudia Valenzuela, Marlies S Wijsenbeek, Donald F Zoz, Florian Voss, Toby M Maher, 1305-0013 Trial Investigators. N Engl J Med. 2022 May 15. doi: 10.1056/NEJMoa2201737. Online ahead of print.
2021
Natural variability in the disease course of SSc-ILD: implications for treatment.
Authors: Madelon C Vonk, Ulrich A Walker, Elizabeth R Volkmann, Michael Kreuter, Sindhu R Johnson, Yannick Allanore. Eur Respir Rev. 2021 Mar 24;30(159):200340.doi: 10.1183/16000617.0340-2020. Print 2021 Mar 31
Interstitial lung diseases in the neonatal period.
Authors: Yohan Soreze, Chiara Sileo, Aurore Coulomb l’Hermine, Marie Legendre, Nadia Nathan. Eur Respir Monogr 2021; 92: 213-230
Authors: Yohan Soreze, Chiara Sileo, Aurore Coulomb l’Hermine, Marie Legendre, Nadia Nathan. Eur Respir Monogr 2021; 92: 213-230
Authors: Yohan Soreze, Chiara Sileo, Aurore Coulomb l’Hermine, Marie Legendre, Nadia Nathan. Eur Respir Monogr 2021; 92: 213-230
Authors: Gizal Nakshbandi, Catharina C. Moor, Kerri A. Johannson, Toby M. Maher, Michael Kreuter, Marlies S. Wijsenbeek. ERJ Open Res, 7 (3) 00405-2021; 10.1183/23120541.00405-2021
Authors: Vincent Cottin, Sophie Larrieu, Loic Boussel, Salim Si-Mohamed, Fabienne Bazin, Sébastien Marque, Jacques Massol, Françoise Thivolet-Bejui, Lara Chalabreysse, Delphine Maucort-Boulch, Stéphane Jouneau, Eric Hachulla, Julien Chollet, Mouhamad Nasser. Front Med (Lausanne). 2021 Aug 30;8:699532. doi: 10.3389/fmed.2021.699532. eCollection 2021.
Authors: Michael Kreuter, Toby M Maher, Tamera J Corte, Maria Molina-Molina, Judit Axmann, Frank Gilberg, Klaus-Uwe Kirchgaessler, Vincent Cottin. Adv Ther. 2021 Dec 22. doi: 10.1007/s12325-021-02009-w. Online ahead of print.
Gender Differences in Idiopathic Pulmonary Fibrosis: Are Men and Women Equal?
Authors: Lucile Sesé, Hilario Nunes, Vincent Cottin, Dominique Israel-Biet, Bruno Crestani, Stephanie Guillot-Dudoret, Jacques Cadranel, Benoit Wallaert, Abdellatif Tazi, Bernard Maître, Gregoire Prévot, Sylvain Marchand-Adam, Sandrine Hirschi, Sandra Dury, Violaine Giraud, Anne Gondouin, Philippe Bonniaud, Julie Traclet, Karine Juvin, Raphael Borie, Zohra Carton, Olivia Freynet, Thomas Gille, Carole Planès, Dominique Valeyre, Yurdagül Uzunhan. Front Med (Lausanne). 2021 Aug 5;8:713698. doi: 10.3389/fmed.2021.713698. eCollection 2021.
Tuberous sclerosis complex for the pulmonologist.
Authors: Yasmine Rebaine, Mouhamad Nasser, Barbara Girerd, Caroline Leroux, Vincent Cottin. Eur Respir Rev. 2021 Aug 3;30(161):200348. doi: 10.1183/16000617.0348-2020. Print 2021 Sep 30.
Care Delivery Models and Interstitial Lung Disease: The Role of the Specialized Center.
Authors: Jolene H Fisher, Vincent Cottin. Clin Chest Med. 2021 Jun;42(2):347-355.doi: 10.1016/j.ccm.2021.03.013.
Interstitial pneumonia with autoimmune features: challenges and controversies.
Authors: John A Mackintosh, Athol U Wells, Vincent Cottin, Andrew G Nicholson, Elisabetta A Renzoni. Eur Respir Rev. 2021 Dec 22;30(162):210177. doi: 10.1183/16000617.0177-2021. Print 2021 Dec 31.
Variability in Global Prevalence of Interstitial Lung Disease.
Authors: Bhavika Kaul, Vincent Cottin, Harold R Collard, Claudia Valenzuela. Front Med (Lausanne). 2021 Nov 4;8:751181. doi: 10.3389/fmed.2021.751181. eCollection 2021.
Misconceptions regarding symptoms of sarcoidosis.
Authors: Marjolein Drent, Ulrich Costabel, Elliott D Crouser, Johan Grunewald, Francesco Bonella. Lancet Respir Med 2021 Aug;9(8):816-818. doi: 10.1016/S2213-2600(21)00311-8. Epub 2021 Jun 30.
Spectral Photon-Counting CT Technology in Chest Imaging.
Authors: Salim Aymeric Si-Mohamed, Jade Miailhes, Pierre-Antoine Rodesch, Sara Boccalini, Hugo Lacombe, Valérie Leitman, Vincent Cottin, Loic Boussel, Philippe Douek. J Clin Med. 2021 Dec 9;10(24):5757. doi: 10.3390/jcm10245757.
Progressive fibrosing interstitial lung disease: a clinical cohort (the PROGRESS® study).
Authors: Nasser M, Larrieu S, Si-Mohamed S, Ahmad K, Boussel L, Brevet M, Chalabreysse L, Fabre C, Marque S, Revel D, Thivolet-Bejui F, Traclet J, Zeghmar S, Maucort-Boulch D, Cottin V
Authors: Cottin V, Bergot E, Bourdin A, Nunes H, Prévot G, Wallaert B, Marchand-Adam S
Epidemiology of systemic sclerosis: a multi-database population-based study in Tuscany (Italy)
Authors: Coi A, Barsotti S, Santoro M, Almerigogna F, Bargagli E, Caproni M, Emmi G, Frediani B, Guiducci S, Matucci Cerinic M, Mosca M, Parronchi P, Prediletto R, Selvi E, Simonini G, Tavoni AG
Authors: Fallerini C, Daga S, Mantovani S, Benetti E, Picchiotti N, Francisci D, Paciosi F, Schiaroli E, Baldassarri M, Fava F, Palmieri M, Ludovisi S, Castelli F, Quiros-Roldan E, Vaghi M, Rusconi S, Siano M, Bandini M, Spiga O, Capitani K, Furini S, Mari F; GEN-COVID Multicenter Study, Renieri A, Mondelli MU, Frullanti E.
2020
Authors: d’Alessandro M, Bergantini L, Refini RM, Cameli P, Perillo F, Landi C, Icorne F, Perrone A, Sestini P, Bonella F, Bargagli E
Desquamative interstitial pneumonia: a systematic review of its features and outcomes
Authors: Hellemons ME, Moor CC, von der Thusen J, Rossius M, Odink A, Thorgersen LH, Verschakelen J, Wuyts W, Wijsenbeek MS, Bendstrup E
Current treatment of sarcoidosis
Authors: Sellares J, Francesqui J, Llabres M, Hernandez-Gonzalez F, Baughman RP
Diagnostic Approach of Fibrosing Interstitial Lung Diseases of Unknown Origin
Authors: Cottin V, Valenzuela C
Authors: Cottin V, Nasser M, Traclet J, Chalabreysse L, Lèbre A-S, Si-Mohamed S, Philit F, Thivolet-Béjui F
Authors: Lancaster LH, Valenzuela C, Mason W, Neurohr C, Ripamonti E, Kirchgaessler K-U, Cottin V
2019
Authors: Cottin V, Annesi-Maesano I, Günther A, Galvin L, Kreuter M, Powell P, Prasse A, Reynolds G, Richeldi L, Spagnolo P, Valenzuela C, Wijsenbeek M, Wuyts WA, Crestani B; Ariane-IPF Clinical Research Collaboration consortium.
Authors: Cavagna L, Trallero-Araguás E, Meloni F, Cavazzana I, Rojas-Serrano J, Feist E, Zanframundo G, Morandi V, Meyer A, Pereira da Silva JA, Matos Costa CJ, Molberg O, Andersson H, Codullo V, Mosca M, Barsotti S, Neri R, Scirè C, Govoni M, Furini F, Lopez-Longo FJ, Martinez-Barrio J, Schneider U, Lorenz HM, Doria A, Ghirardello A, Ortego-Centeno N, Confalonieri M, Tomietto P, Pipitone N, Rodriguez Cambron AB, Blázquez Cañamero MÁ, Voll RE, Wendel S, Scarpato S, Maurier F, Limonta M, Colombelli P, Giannini M, Geny B, Arrigoni E, Bravi E, Migliorini P, Mathieu A, Piga M, Drott U, Delbrueck C, Bauhammer J, Cagnotto G, Vancheri C, Sambataro G, De Langhe E, Sainaghi PP, Monti C, Gigli Berzolari F, Romano M, Bonella F, Specker C, Schwarting A, Villa Blanco I, Selmi C, Ceribelli A, Nuno L, Mera-Varela A, Perez Gomez N, Fusaro E, Parisi S, Sinigaglia L, Del Papa N, Benucci M, Cimmino MA, Riccieri V, Conti F, Sebastiani GD, Iuliano A, Emmi G, Cammelli D, Sebastiani M, Manfredi A, Bachiller-Corral J, Sifuentes Giraldo WA, Paolazzi G, Saketkoo LA, Giorgi R, Salaffi F, Cifrian J, Caporali R, Locatelli F, Marchioni E, Pesci A, Dei G, Pozzi MR, Claudia L, Distler J, Knitza J, Schett G, Iannone F, Fornaro M, Franceschini F, Quartuccio L, Gerli R, Bartoloni E, Bellando Randone S, Zampogna G, Gonzalez Perez MI, Mejia M, Vicente E, Triantafyllias K, Lopez-Mejias R, Matucci-Cerinic M, Selva-O’Callaghan A, Castañeda S, Montecucco C, Gonzalez-Gay MA.
Research highlights from the 2018 ERS International Congress: interstitial lung diseases.
Authors: Alfaro TM, Moor CC, Alfieri V, Jeny F, Kreuter M, Wijsenbeek MS, Renzoni EA, Bargagli E, Nunes H, Spagnolo P, Bonella F, Molina-Molina M, Antoniou K, Poletti V.
2018
The European research collaboration for Children’s Interstitial Lung Disease (ChILDEU) ERS Clinical Research Collaboration.
Authors: Cunningham S, Gilbert C, Schwerk N; ChILDEU Clinical Research Collaboration Management Committee; Members of the ChILDEU Clinical Research Collaboration Management Committee.
Authors: Sara Naranjo Gozalo; María de los Ángeles Ballesteros Sanz; Carlos Álvarez De Arriba; Víctor Manuel Mora Cuesta; Eduardo Miñambres García; Laura Sánchez Moreno. Journal of Visualized Experiments. 15/08/2022. DOI: 10.3791/63975
Antifibrotics and lung transplantation: A Spanish multicentre case-controlled study.
Authors: Víctor Manuel Mora Cuesta; David Iturbe Fernández; Silvia Aguado Ibáñez; Gabriel Anguera De Francisco; Juan Margallo Iribarnegaray; Javier Carrillo Hernández Rubio; Juan Pablo Reig Mezquida; Virginia Pérez Luz; Rosalía Laporta Hernández; Alicia De Pablo Gafas; Amparo Solé Jover; José Manuel Cifrián MartínezRespirology. 27, Wiley, 28/08/2022. DOI: 0.1111/resp.14352
2025
Meta-analysis of [18F]FDG-PET/CT in pulmonary sarcoidosis.
Authors: Donnelly R, McDermott M, McManus G, Franciosi AN, Keane MP, McGrath EE, McCarthy C, Murphy DJ. Eur Radiol. 35/2025. doi: 10.1007/s00330-024-10949-4.
Multinational Survey on the Symptom Approach to Diagnosis and Therapy Adopted by Physicians in the Treatment of Gastrointestinal Sarcoidosis.
Authors: Salvatore Nicolosi, Giorgio Monteleone, Giulia Bandini, Michael Hughes, Zsuzsanna McMahan, Elena Bargagli, Filippo Martone, Gianluca Ziosi, Paola Confalonieri, Marco Confalonieri, Paolo Cameli, Barbara Ruaro. J Clin Med. 11/2025. doi: 10.3390/jcm14228231.
Serum KL-6 as a biomarker to predict progression at one year in interstitial lung disease.
Authors: Francesco Bonella, M C Vegas Sanchez, Miriana d’Alessandro, P Millan-Billi, R F Santos, N Schröder, H N Bastos, Maria Molina-Molina, O Sánchez Pernaute, Diogo Castillo Villegas, Elena Bargagli. Sci Rep. 10/2025. doi: 10.1038/s41598-025-22483-4.
Predictive role of natural killer cells in bronchoalveolar lavage fluid of patients with sarcoidosis.
Authors: Laura Bergantini, Marco Spalletti, Miriana d’Alessandro, Michele Genovese, Elena Masotto, Paolo Cameli, Antje Prasse, Elena Bargagli. Pulmonology. 12/2025. doi: 10.1016/j.pulmoe.2023.11.009.
2024
Kidney manifestations of sarcoidosis.
Bonella F, Dm Vorselaars A, Wilde B.J Autoimmun. 2024 Dec;149:103207. doi: 10.1016/j.jaut.2024.103207. Epub 2024 Mar 22.PMID: 38521611 Free article. Review.
Online survey on existing sarcoidosis registries and biobanks: an ERN lung initiative.
D’alessandro M, Pianigiani T, Bergantini L, Cameli P, Bargagli E, Balestro E, Börner E, Bonella F.Sarcoidosis Vasc Diffuse Lung Dis. 2024 Dec 10;41(4):e2024063. doi: 10.36141/svdld.v41i4.16074.PMID: 39655584
The management of Familial Pulmonary Fibrosis in different medical settings: Where does that leave us? An Italian nationwide survey.
Authors: Monteleone G, ILDs Study Group Sip/Irs, Bergantini L, D’Alessandro M, Pianigiani T, Simonetti J, Iovene B, Varone F, Sgalla G, Richeldi L, Bargagli E, Cameli P. Sarcoidosis Vasc Diffuse Lung Dis. 2024 Sep 24;41(3):e2024047. doi: 10.36141/svdld.v41i3.15744.
Predictive role of natural killer cells in bronchoalveolar lavage fluid of patients with sarcoidosis
Authors: Bergantini L, Spalletti M, d’Alessandro M, Genovese M, Masotto E, Cameli P, Prasse A, Bargagli E.. Pulmonology. 2025 Dec 31;31(1):2416867. doi:10.1016/j.pulmoe.2023.11.009. Epub 2024 Oct 25.
The healthcare burden of pulmonary alveolar proteinosis (PAP).
Authors: Lee E, Ataya A, McCarthy C, Godart E, Cosenza J, King A, Robinson B, Wang T. Orphanet J Rare Dis. 2025 Feb 14;20(1):73. doi: 10.1186/s13023-024-03478-2. PMID: 39953603.
Risk of lung disease with the Pi*SS genotype of alpha-1 antitrypsin: the evidence in context.
Authors: O’Brien H, McCarthy C, Franciosi AN. Respir Res. 2025 Jan 24;26(1):37. doi: 10.1186/s12931-024-03066-9. PMID: 39856741
Does ATP via purinergic receptor signalling fuel pulmonary fibrosis?
Authors: Forde L, Gogoi D, Baird R, McCarthy C, Keane MP, Reeves EP, McGrath EE. Journal of Innate Immunity. 2025;17(1):44-55. doi: 10.1159/000543083. Epub 2024 Dec 11. PMID: 39662078; PMCID: PMC11779128.
Meta-analysis of 18F-FDG-PET/CT in pulmonary sarcoidosis
Authors: Donnelly R, McDermott M, McManus G, Franciosi AN, Keane MP, McGrath EE, McCarthy C*, Murphy DJ*.. Eur Radiol. 2024 Jul 23. doi: 10.1007/s00330-024-10949-4. Epub ahead of print. PMID: 39044038.
Unsupervised Exercise in Interstitial Lung Disease: A Delphi Study to Develop a Consensus Preparticipation Screening Tool for Lymphangioleiomyomatosis.
Authors: Child CE, Ho LA, Lachant D, Gupta N, Moss J, Jones A, Krishna R, Holland AE, Han MK, McCarthy C, Ataya A, Baqir M, Dilling D, Swigris J, Swenson ER, Brown MB. CHEST 2024. Jul 16:S0012-3692(24)04613-0. doi: 10.1016/j.chest.2024.06.3803. Epub ahead of print. PMID: 39025205.
Northern European LAM Prevalence Consortium. Updated Prevalence of Lymphangioleiomyomatosis in Europe.
Authors: Lynn E, Forde SH, Franciosi AN, Bendstrup E, Veltkamp M, Wind AE, Van Moorsel CHM, Lund TK, Durheim MT, Peeters EFHI, Keane MP, McCarthy C; and Am J Respir Crit Care Med. 2024 Feb 15;209(4):456-459. doi: 10.1164/rccm.202310-1736LE. PMID: 38060201.
Re-examining the role of pulmonary lipids in the pathogenesis of pulmonary fibrosis.
Authors: O’Callaghan M, Tarling EJ, Bridges JP, Redente EF, Byrne AJ, Keane MP, McCarthy C Am J Respir Cell Mol Biol. 2024. May 31. doi: 10.1165/rcmb.2024-0124PS. Epub ahead of print. PMID: 38820234.
Autoimmune Pulmonary Alveolar Proteinosis: A Review of Pathogenesis and Emerging Therapies
Authors: Mathavan A, Mathavan A, Sathyanarayanan SP, McCarthy C, Ataya A.. Curr Pulmonol Rep. 2024. https://doi.org/10.1007/s13665-024-00356-x
Pulmonary Manifestations of Sjögren’s Disease.
Authors: Byrne L, McCarthy C*, Fabre A. Gupta N. Semin Respir Crit Care Med 2024. Jun;45(3):397-410. doi: 10.1055/s-0044-1785675. Epub 2024 Apr 15. PMID: 38621712.
Kidney manifestations of sarcoidosis.
Bonella F, Dm Vorselaars A, Wilde B.J Autoimmun. 2024 Dec;149:103207. doi: 10.1016/j.jaut.2024.103207. Epub 2024 Mar 22.PMID: 38521611 Free article. Review.
Online survey on existing sarcoidosis registries and biobanks: an ERN lung initiative.
D’alessandro M, Pianigiani T, Bergantini L, Cameli P, Bargagli E, Balestro E, Börner E, Bonella F.Sarcoidosis Vasc Diffuse Lung Dis. 2024 Dec 10;41(4):e2024063. doi: 10.36141/svdld.v41i4.16074.PMID: 39655584
The management of Familial Pulmonary Fibrosis in different medical settings: Where does that leave us? An Italian nationwide survey.
Authors: Monteleone G, ILDs Study Group Sip/Irs, Bergantini L, D’Alessandro M, Pianigiani T, Simonetti J, Iovene B, Varone F, Sgalla G, Richeldi L, Bargagli E, Cameli P. Sarcoidosis Vasc Diffuse Lung Dis. 2024 Sep 24;41(3):e2024047. doi: 10.36141/svdld.v41i3.15744.
Predictive role of natural killer cells in bronchoalveolar lavage fluid of patients with sarcoidosis
Authors: Bergantini L, Spalletti M, d’Alessandro M, Genovese M, Masotto E, Cameli P, Prasse A, Bargagli E.. Pulmonology. 2025 Dec 31;31(1):2416867. doi:10.1016/j.pulmoe.2023.11.009. Epub 2024 Oct 25.
The healthcare burden of pulmonary alveolar proteinosis (PAP).
Authors: Lee E, Ataya A, McCarthy C, Godart E, Cosenza J, King A, Robinson B, Wang T. Orphanet J Rare Dis. 2025 Feb 14;20(1):73. doi: 10.1186/s13023-024-03478-2. PMID: 39953603.
Risk of lung disease with the Pi*SS genotype of alpha-1 antitrypsin: the evidence in context.
Authors: O’Brien H, McCarthy C, Franciosi AN. Respir Res. 2025 Jan 24;26(1):37. doi: 10.1186/s12931-024-03066-9. PMID: 39856741
Does ATP via purinergic receptor signalling fuel pulmonary fibrosis?
Authors: Forde L, Gogoi D, Baird R, McCarthy C, Keane MP, Reeves EP, McGrath EE. Journal of Innate Immunity. 2025;17(1):44-55. doi: 10.1159/000543083. Epub 2024 Dec 11. PMID: 39662078; PMCID: PMC11779128.
Meta-analysis of 18F-FDG-PET/CT in pulmonary sarcoidosis
Authors: Donnelly R, McDermott M, McManus G, Franciosi AN, Keane MP, McGrath EE, McCarthy C*, Murphy DJ*.. Eur Radiol. 2024 Jul 23. doi: 10.1007/s00330-024-10949-4. Epub ahead of print. PMID: 39044038.
Unsupervised Exercise in Interstitial Lung Disease: A Delphi Study to Develop a Consensus Preparticipation Screening Tool for Lymphangioleiomyomatosis.
Authors: Child CE, Ho LA, Lachant D, Gupta N, Moss J, Jones A, Krishna R, Holland AE, Han MK, McCarthy C, Ataya A, Baqir M, Dilling D, Swigris J, Swenson ER, Brown MB. CHEST 2024. Jul 16:S0012-3692(24)04613-0. doi: 10.1016/j.chest.2024.06.3803. Epub ahead of print. PMID: 39025205.
Northern European LAM Prevalence Consortium. Updated Prevalence of Lymphangioleiomyomatosis in Europe.
Authors: Lynn E, Forde SH, Franciosi AN, Bendstrup E, Veltkamp M, Wind AE, Van Moorsel CHM, Lund TK, Durheim MT, Peeters EFHI, Keane MP, McCarthy C; and Am J Respir Crit Care Med. 2024 Feb 15;209(4):456-459. doi: 10.1164/rccm.202310-1736LE. PMID: 38060201.
Re-examining the role of pulmonary lipids in the pathogenesis of pulmonary fibrosis.
Authors: O’Callaghan M, Tarling EJ, Bridges JP, Redente EF, Byrne AJ, Keane MP, McCarthy C Am J Respir Cell Mol Biol. 2024. May 31. doi: 10.1165/rcmb.2024-0124PS. Epub ahead of print. PMID: 38820234.
Autoimmune Pulmonary Alveolar Proteinosis: A Review of Pathogenesis and Emerging Therapies
Authors: Mathavan A, Mathavan A, Sathyanarayanan SP, McCarthy C, Ataya A.. Curr Pulmonol Rep. 2024. https://doi.org/10.1007/s13665-024-00356-x
Pulmonary Manifestations of Sjögren’s Disease.
Authors: Byrne L, McCarthy C*, Fabre A. Gupta N. Semin Respir Crit Care Med 2024. Jun;45(3):397-410. doi: 10.1055/s-0044-1785675. Epub 2024 Apr 15. PMID: 38621712.
2023
Images in Sarcoidosis: new section of our Journal.
Authors: Baughman RP, Caminati A, Ravaglia C, Richeldi L, Rottoli P, Tomassetti S, Vancheri C. Sarcoidosis Vasc Diffuse Lung Dis. 2023 Dec 20;40(4):e2023047. doi: 10.36141/svdld.v40i4.15339. PMID: 38126493
Peripheral Airway Sarcoidosis: Seeing What Was Previously Invisible.
Authors: Trisolini R, Pasciuto G, Cancellieri A, Agrosì C, Magnini D, Richeldi L. Am J Respir Crit Care Med. 2023 Dec 15;208(12):1328-1329. doi: 10.1164/rccm.202304-0781IM. PMID: 37642604 No abstract available.
High-resolution CT phenotypes in pulmonary sarcoidosis: a multinational Delphi consensus study.
Authors: Desai SR, Sivarasan N, Johannson KA, George PM, Culver DA, Devaraj A, Lynch DA, Milne D, Renzoni E, Nunes H, Sverzellati N, Spagnolo P, Baughman RP, Yadav R, Piciucchi S, Walsh SLF, Kouranos V, Wells AU; Sarcoid Delphi Group. Lancet Respir Med. 2023 Dec 14:S2213-2600(23)00267-9. doi: 10.1016/S2213-2600(23)00267-9. Online ahead of print. PMID: 38104579 Review.
High- resolution CT phenotypes in pulmonary sarcoidosis: a multinational Delphi consensus study.
Authors: Desai SR, Sivarasan N, Johannson KA, George PM, Culver DA, Devaraj A, Lynch DA, Milne D, Renzoni E, Nunes H, Sverzellati N, Spagnolo P, Baughman RP, Yadav R, Piciucchi S, Walsh SLF, Kouranos V, Wells AU; Sarcoid Delphi Group. Lancet Respir Med. 2023 Dec 14:S2213-2600(23)00267-9. doi: 10.1016/S2213-2600(23)00267-9. Epub ahead of print. PMID: 38104579.
Genetic and geographic influence on phenotypic variation in European sarcoidosis patients.
Authors: Freitag-Wolf S, Schupp JC, Frye BC, Fischer A, Anwar R, Kieszko R, Mihailović-Vučinić V, Milanowski J, Jovanovic D, Zissel G, Bargagli E, Rottoli P, Bumbacea D, Jonkers R, Ho LP, Gaede KI, Dubaniewicz A, Marshall BG, Günther A, Petrek M, Keane MP, Haraldsdottir SO, Bonella F, Grah C, Peroš-Golubičić T, Kadija Z, Pabst S, Grohé C, Strausz J, Safrankova M, Millar A, Homolka J, Wuyts WA, Spencer LG, Pfeifer M, Valeyre D, Poletti V, Wirtz H, Prasse A, Schreiber S, Dempfle A, Müller-Quernheim J. Front Med (Lausanne). 2023 Aug 9;10:1218106. doi: 10.3389/fmed.2023.1218106. PMID: 37621457; PMCID: PMC10446882.
Thoracic pain in patients with chronic interstitial lung disease-an underestimated symptom.
Authors: Scherer MJ, Kampe S, Fredebeul-Beverungen J, Weinreich G, Costabel U, Bonella F. Front Med (Lausanne). 2023 May 5;10:1147555. doi: 10.3389/fmed.2023.1147555. PMID: 37215705; PMCID: PMC10196162.
Therapie der Sarkoidose. Ein Positionspapier der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin (DGP) [Therapeutic Pathways in Sarcoidosis. A Position Paper of the German Society of Respiratory Medicine (DGP)].
Authors: Skowasch D, Bonella F, Buschulte K, Kneidinger N, Korsten P, Kreuter M, Müller-Quernheim J, Pfeifer M, Prasse A, Quadder B, Sander O, Schupp JC, Sitter H, Stachetzki B, Grohé C. Pneumologie. 2024 Mar;78(3):151-166. German. doi: 10.1055/a-2259-1046. Epub 2024 Feb 26. PMID: 38408486.”
High- resolution CT phenotypes in pulmonary sarcoidosis: a multinational Delphi consensus study.
Authors: Desai SR, Sivarasan N, Johannson KA, George PM, Culver DA, Devaraj A, Lynch DA, Milne D, Renzoni E, Nunes H, Sverzellati N, Spagnolo P, Baughman RP, Yadav R, Piciucchi S, Walsh SLF, Kouranos V, Wells AU; Sarcoid Delphi Group. Lancet Respir Med. 2023 Dec 14:S2213-2600(23)00267-9. doi: 10.1016/S2213-2600(23)00267-9. Epub ahead of print. PMID: 38104579.
Genetic and geographic influence on phenotypic variation in European sarcoidosis patients.
Authors: Freitag-Wolf S, Schupp JC, Frye BC, Fischer A, Anwar R, Kieszko R, Mihailović-Vučinić V, Milanowski J, Jovanovic D, Zissel G, Bargagli E, Rottoli P, Bumbacea D, Jonkers R, Ho LP, Gaede KI, Dubaniewicz A, Marshall BG, Günther A, Petrek M, Keane MP, Haraldsdottir SO, Bonella F, Grah C, Peroš-Golubičić T, Kadija Z, Pabst S, Grohé C, Strausz J, Safrankova M, Millar A, Homolka J, Wuyts WA, Spencer LG, Pfeifer M, Valeyre D, Poletti V, Wirtz H, Prasse A, Schreiber S, Dempfle A, Müller-Quernheim J. Front Med (Lausanne). 2023 Aug 9;10:1218106. doi: 10.3389/fmed.2023.1218106. PMID: 37621457; PMCID: PMC10446882.
Reply to letter to the Editor: Current role of [18F] FDG-PET/CT in pulmonary sarcoidosis: a meta-analysis.
Authors: Donnelly R, McCarthy C, Murphy DJ. Eur Radiol. 2024 Dec 3. doi: 10.1007/s00330-024-11194-5. Epub ahead of print. PMID: 39625503.
Lymphangioleiomyomatosis and its Treatment: When to Start?
Authors: O’Malley D, Byrne AJ, Keane MP, McCarthy C. Arch Bronconeumol. 2024 Sep 24:S0300-2896(24)00365-X. doi:10.1016/j.arbres.2024.09.005. Epub ahead of print. PMID: 39366813.
Alveolar macrophage lipid burden correlates with clinical improvement in patients with pulmonary alveolar proteinosis.
Authors: Lee E, Williams KJ, McCarthy C, Bridges JP, Redente EF, de Aguiar Vallim TQ, Barrington RA, Wang T, Tarling EJ. J Lipid Res. 2024 Feb;65(2):100496. doi: 10.1016/j.jlr.2024.100496. Epub 2024 Jan 6. PMID: 38185217; PMCID: PMC10844116.
Diffuse Cystic Lung Disease: A Clinical Guide to Recognition and Management.
Authors: Franciosi AN, Gupta N, Murphy DJ, Wikenheiser-Brokamp KA, McCarthy C. CHEST. 2024. 2024 Aug 19:S0012-3692(24)04923-7. doi: 10.1016/j.chest.2024.08.008. Epub ahead of print. PMID: 39168181.
ERN GENTURIS clinical practice guidelines for the diagnosis, surveillance and management of people with Birt-Hogg-Dubé syndrome.
Authors: Geilswijk M, Genuardi M, Woodward ER, Nightingale K, Huber J, Madsen MG, Liekelema-van der Heij D, Lisseman I, Marlé-Ballangé J, McCarthy C, Menko FH, Moorselaar RJAV, Radzikowska E, Richard S, Rajan N, Sommerlund M, Wetscherek MTA, Di Donato N, Maher ER, Brunet J. Eur J Hum Genet. 2024 Jul 31. doi: 10.1038/s41431-024-01671-2. Epub ahead of print. PMID: 39085584.
2022
Sarkoidose
Authors: J. Wälscher, T.e.Wessendorf, Kaid Darwiche, Francesco Bonella. April 2022/ Article /Pneumologie76(04):281-293 ; DOI:1055/a-1275-4838
Prevalence and quantification of thoracic pain (TP) through quantitative sensory testing (QST) in patients with chronic interstitial lung disease (cILD)
Authors: M.J. Scherer , S. Kampe, J. Fredebeul-Beverungen, F. Bonella / PrePrint /DOI:21203/rs.3.rs-1415115/v1
Pulmonale Alveolarproteinose
Authors: F. Bonella, N. Lachmann, M. Kreuter, U. Costabel / Chapter / DOI:1007/978-3-662-63651-0_20. In book: Seltene Lungenerkrankungen (pp.303-315), Feb 2022
Pulmonary sarcoidosis
Authors: W.E. James, F. Bonella / Chapter /DOI:1183/2312508X.10031820 ; In book: Sarcoidosis (pp.122-141), March 2022
Targeted therapy for pulmonary alveolar proteinosis: the time is now
Authors: F. Bonella, R. Borie / Article / April 2022, European Respiratory Journal 59(4):2102971DOI:10.1183/13993003.02971-2021
Effects of nintedanib in patients with idiopathic pulmonary fibrosis and varying severities of cough*
Authors: F.Bonella, W.A. Wuyts , C. Vancheri, M. Wijsenbeek / Conference Paper / / May 2022, Pneumologie76 ; DOI:1055/s-0042-1747732
Pulmonale Manifestationen rheumatologischer Erkrankungen
Authors: M. Kreuter, H.-M-Lorenz, F. Bonella, A.-M.Hoffmann-Vold, Feb 2022 / Chapter / DOI:1007/978-3-662-63651-0_8. In book: Seltene Lungenerkrankungen (pp.83-100).
Effects of Nintedanib on Circulating Biomarkers in Subjects with Progressive Fibrosing Interstitial Lung Diseases (ILDs)
Authors: T.M. Maher, G. Jenkins, F. Bonella, on behalf oft he INBUILD trial investigators / Conference Paper / /May 2022 ,DOI:1164/ajrccm-conference.2022.205.1_MeetingAbstracts.A3520
ORMDL3 Gene Polymorphisms in Patients with Pulmonary Alveolar Proteinosis (PAP) and Interstitial Lung Diseases (ILDs): Preliminary Results
Authors: F. Bonella D. Hässig, S. Cabric, M. Schedel / Conference Paper, / May 2022 , / DOI:1164/ajrccm-conference.2022.205.1_MeetingAbstracts.A3922
Efficacy and Safety of Nintedanib in Elderly Patients with Progressive Fibrosing Interstitial Lung Diseases (ILDs)
Authors: T. Moua, F. Bonella, M. Okamoto, on behalf oft he INBUILD trial investigators / May 2022, Conference Paper /
DOI:1164/ajrccm-conference.2022.205.1_MeetingAbstracts.A2426
Vergleich einer neuen Stanznadel mit einer herkömmlichen 22-Gauge Nadel im Rahmen der EBUS-TBNA bei Patienten mit Verdacht auf Sarkoidose
Authors: E. Büscher, Kaid Darwiche, F. Bonella, J. Wälscher / May 2022, Conference Paper / Pneumologie76DOI:10.1055/s-0042-1747789
Immuncheckpoint-Inhibitor (ICI)-assoziierte Lungenmanifestationen (ICI-LM): Charakterisierung der HRCT- und BAL-Befunde
Authors: L.B. Jehn, J. Wälscher, T.E: Wessendorf, F. Bonella / Conference Paper // May 2022, Pneumologie76 ; DOI:1055/s-0042-1747806
ORMDL3 gene polymorphisms in patients with pulmonary alveolar proteinosis (PAP) and interstitial lung diseases (ILDs): preliminary results.
Authors: F. Bonella, D. Hässig, S. Cabric, M. Schedel /May 2022, Conference Paper / Pneumologie76 ; DOI:1055/s-0042-1747814
Continued nintedanib treatment in patients with progressive fibrosing ILDs: interim analysis of INBUILD-ON*
Authors: F. Bonella, W.A. Wuyts, N. Chaudhuri, F. Varone / Conference Paper / Pneumologie76 ; DOI:1055/s-0042-1747743
Meta-Analysis of Effect of Nintedanib on Reducing FVC Decline Across Interstitial Lung Diseases
Authors: F. Bonella, V. Cottin C. Valenzuela, T.M. Maher / Article / May 2022, Advances in Therapy39(7) DOI:10.1007/s12325-022-02145-x
IL-9 and IL-9 receptor expression in lymphocytes from bronchoalveolar lavage fluid of patients with interstitial lung disease
Authors: L.B. Jehn, U. Costabel, Eda Burcu Börner, F. Bonella / Aug. 2022, Article / Immunobiology227(5):152258 ;DOI:1016/j.imbio.2022.152258
Nintedanib in patients with progressive fibrosing ILDs in subgroups by comorbidity burden
Authors: E. Bendstrup, T. Moua, M. Okamoto , F. Bonella / Conference Paper /Dec. 2022, DOI:1183/13993003.congress-2022.615 ;Conference: ERS International Congress 2022 abstracts
Serum KL-6 improves prediction of progressive phenotype (PP) at one year independently of underlying interstitial lung disease (ILD): results from the VAMOS study.
Authors: F. Bonella, M.C. Vegas Sanchez , M. Molina Molina / Conference Paper, / Dec. 2022, /DOI:1183/13993003.congress-2022.1274 ;Conference: ERS International Congress 2022 abstracts
Continued nintedanib treatment in patients with progressive fibrosing ILDs: interim analysis of INBUILD-ON
Authors: W.A. Wuyts, F. Bonella, N. Chaudhuri, V. Cottin / Dec. 2022, Conference Paper / DOI:1183/13993003.congress-2022.604 ;Conference: ERS International Congress 2022 abstracts
Comparison of a 22G Crown-Cut Needle with a Conventional 22G Needle with EBUS Guidance in Diagnosis of Sarcoidosis
Authors: J. Wälscher, E. Büscher, F. Bonella, K. Darwiche / Sept. 2022, Article /Lung 200(3) ;DOI:1007/s00408-022-00562-x
2021
S2K Guideline for Diagnosis of Idiopathic Pulmonary Fibrosis
Authors: J. Behr, A. Günther, F. Bonella, U. Costabel / Respiration100(3):1-34 ; / Jan. 2021, DOI:1159/000512315
Effects of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis and Varying Severities of Cough
Authors: W.A. Wuyts, C. Vancheri, F. Bonela, M.S.Wijsenbeek / Conference Paper, / May 2021
Potential clinical utility of MUC5B und TOLLIP single nucleotide polymorphisms (SNPs) in the management of patients with IPF / Feb. 2021 , F. Bonella, I. Campo, M. Zorzetto, U. Costabel / Orphanet Journal of Rare Diseases16(1):111 DOI:1186/s13023-021-01750-3
Efficacy and safety of nintedanib in patients with idiopathic pulmonary fibrosis who are elderly or have comorbidities
Authors: Ian Glaspole, Francesco Bonella, Elena Bargagli, Marilyn k. Glassberg / April 2021 /Respiratory Research22(1) DOI:1186/s12931-021-01695-y
Misconceptions regarding symptoms of sarcoidosis
Authors: M.Drent, U. Costabel, E.D. Crouser, F. Bonella /June 2021, The Lancet Respiratory Medicine9(8) DOI:1016/S2213-2600(21)00311-8
POS0834 LONG-TERM OUTCOME OF SSC ASSOCIATED ILD: IMPROVED SURVIVAL IN PPI TREATED PATIENTS
Authors: M. Kreuter , F. Bonella, K. Kathrin, J. Henes / Annals of the Rheumatic Diseases 80(Suppl 1):670.2-671, / June 2021, DOI:1136/annrheumdis-2021-eular.878
Unclassifiable, or simply unclassified interstitial lung disease?
Authors: M. O`Callaghan, F. Bonella, C. McCarthy /Current Opinion in Pulmonary Medicine Publish Ahead of Print(5), June 2021, DOI:10.1097/MCP.0000000000000801
Continued nintedanib treatment in patients with progressive fibrosing ILDs: interim analysis of INBUILD-ON
Authors: W.A. Wuyts, F. Bonella, N. Chaudhuri, V. Cottin / Conference Paper, Sep. 2021,
Serum KL-6 as a biomarker to assess risk of worse outcome in patients with SARS-CoV2 infection
Authors: S. Sutharsan, M. Berger, J. Andrä, F. Bonella / Conference Paper, Sep. 2021
Modified GAP index by including serum KL-6 to assess risk of disease progression in patients with interstitial lung diseases (ILD): preliminary results from the VAMOS study Sept. 2021
Authors: F. Bonella, M.C. Vegas Sanchez , P. Millan. E. Gottstein / Conference Paper
Bronchoalveolar lavage fluid (BALF) findings in patients with immune checkpoint inhibitor (ICI)-associated interstitial pneumonia (IP)
Authors: L.B. Jehn, J. Wälscher, T.E.Wessendorf, F. Bonella / Conference Paper, / Sept. 2021
Prevalence and quantification of thoracic pain (TP) through quantitative sensory testing (QST) in patients with chronic interstitial lung disease (cILD)
Authors: M.Scherer, S. Kampe, U. Costabel, F. Bonella / Conference Paper, Sept. 2021,
Comparison of a 22G crown-cut needle with a conventional 22G needle with EBUS guidance in diagnosis of sarcoidosis
Authors: E. Büscher, Kaid Darwiche , F. Bonella, J. Wälscher / Conference Paper/ Sept. 2021
Idiopathic pulmonary fibrosis: Physician and patient perspectives on the pathway to care from symptom recognition to diagnosis and disease burden
Authors: L. Lancaster, F. Bonela, Y. Inoue, J. Langgley / Respirology27(1), Oct. 2021, ;DOI:1111/resp.14154
Communicating with patients with IPF: can we do it better?
Authors: M. Wijsenbeek, F. Bonella, L. Orsatti, W.F. Baile / Nov. 2021, ERJ Open Research 8(1):00422-2021, DOI:10.1183/23120541.00422-2021
Beruflich bedingte EAA und die problematische Suche nach dem Auslöser
Authors: H. Knoop, U. Knoop, F. Bonella / Dec. 2021, Atemwegs- und Lungenkrankheiten 47(12):578-582, DOI:10.5414/ATX02581
2020
Effects of nintedanib in patients with progressive fibrosing ILDs and differing baseline FVC: further analyses of the INBUILD trial
Authors: Claudia Valenzuela, Toby M. Maher, Francesco Bonella, Vincent Cottin / keine Angabe, Jan 2020
Efficacy and safety of nintedanib in the elderly patient with IPF*
Authors: Francesco Bonella, Elisabeth Bendstrup , Elena, Bargagli, I. Glaspole / Conference Paper, / Mar.2020
Long term outcomes of immunmodulatory drugs in SSc-ILD – data rom the German SSc network
Authors: M. Kreuter, Francesco Bonella, N. Blank, N. Hunzelmann / Conference Paper/ March 2020
Does anti-acid treatment influence disease progression in SSc-ILD? Data form the German SSc-network
Authors: M. Krueter, F. Bonella, N.Blank, N. Hunzelmann / Conference Pape/ March 2020
Klinische Nutzbarkeit eines mit Serum KL-6 modifizierten GAP-Indexes bei Patienten mit interstitiellen Lungenerkrankungen (ILD)
Authors: F. Bonella, B. Hegedues, E. Gottstein, U.Costabel / Conference Paper, March 2020
The INBUILD Trial of Nintedanib in Patients with Progressive Fibrosing Instititial Lung Diseases: Subgroup with Autoimmune Diseases*
Authors: F. Bonella, E. Matteson, Clive Kelly, KR Flaherty / Conference Paper, / March 2020
Berufliche bedingte EAA und die problematische Suche nach dem Auslöser
Authors: Heiko Knoop, Ute Knoop, Francesco Bonella / Conference Paper/ March 2020
Cytokines, chemokines and growth factors concentration in BAL fluid from patients with idiopathic pulmonary fibrosis (IPF) and nonspecific interstitial pneumonia (NSIP)
Authors: Francesco Bonella, David Lamb, Eda Burcu Börner, M. Kaestle / Conference Paper, March 2020
Therapie von Komplikationen und nichtpharmakologisches Management der idiopathischen pulmonalen FibroseTreatment of complications and nonpharmacological management of idiopathic pulmonary fibrosis
Authors: S. Gläser, R.Gloeckl, F. Bonella / March 2020, /Zeitschrift für Pneumologie17(9) DOI:1007/s10405-020-00313-w
S2K-Leitlinie zur Diagnostik der idiopathischen Lungenfibrose
Authors: J. Behr, A. Günther, F. Bonella, U. Costabel / March 2020 Pneumologie74(5) DOI:1055/a-1120-3531
Genotype in MUC5B-Promoter Polymorphism and Chronic Lung Allograft Dysfunction (CLAD) in a German Cohort: Preliminary Results
Authors: S. Krebs, V. Besa, Clemens Aigner, Francesco Bonella // April 2020 The Journal of Heart and Lung Transplantation39(4):S305-S306 DOI:10.1016/j.healun.2020.01.684
Efficacy, safety, and tolerability of combined pirfenidone and N-acetylcysteine therapy: A systematic review and meta-analysis
Authors: Hanyu Shi , Dawei Yin , Francesco Bonella, Luqing Wie // May 2020, BMC Pulmonary Medicine20(1) DOI:1186/s12890-020-1121-2
Azathioprine for connective tissue disease-associated interstitial lung disease (CTD-ILD)
Authors: Francesco Bonella /May 2020 Respiration 99(8) DOI:1159/000508540
Does Anti-Acid Treatment Influence Disease Progression In Systemic Sclerosis Interstitial Lung Disease (Ssc-Ild)? Data From The German Ssc-Network
Authors: M. Kreuter, F. Bonela, G. Riemekasten, N. Hunzelmann / Conference Paper, / July 2020
Looking into the future of sarcoidosis: what is next for treatment?
Authors: J.R. Miedema , F. Bonella, J. Grunewald, P. Spagnolo / July 2020 / Current Opinion in Pulmonary MedicinePublish Ahead of Print(5) DOI:1097/MCP.0000000000000709
The perpetual enigma of bronchoalveolar lavage fluid lymphocytosis in chronic hypersensitivity pneumonitis: Is it of diagnostic value?
Authors: F. Bonella, U. Costabel /Aug. 2020 European Respiratory Journal56(2):2001534 DOI:1183/13993003.01534-2020
Idiopathic pulmonary fibrosis (IPF): the patient journey
Authors: V.Cottin, F. Bonella, M. Small, L. Lancaster / Conference Paper, Sep. 2020
Clinical burden of idiopathic pulmonary fibrosis (IPF): physician and patient perception
Authors: F. Bonella, Y. Inoue, M. Small, L.Lancaster / Conference Paper, Sept. 2020
Effects of nintedanib in patients with progressive fibrosing ILDs and differing baseline FVC: further analyses of the INBUILD trial
Authors: C. Valenzuela, Toby M.Maher, F. Bonella, V. Cottin / Conference Paper, Sept. 2020
Consistent effect of nintedanib on reducing FVC decline across interstitial lung diseases (ILDs)
Authors: F. Bonella, T.M.Maher, V. Cottin, S. Stowasser / Conference Paper, Sept. 2020
Effects of nintedanib in patients with IPF: subgroup analysis by Charlson Comorbidity Index (CCI)
Authors: E. Bendstrup, F. Bonella, E. Bargagli, I. Glaspole / Conference Paper / Sept. 2020
Cytokines, chemokines and growth factors concentration in BAL fluid from patients with Idiopathic pulmonary fibrosis (IPF) and nonspecific interstitial pneumonia (NSIP)
Authors: Marc Kaestle , K.E. Geillinger – Kästle, D. Lamb, Francesco Bonella / Conference Paper/ Sept. 2020
The Gender-Age-Physiology (GAP) Index enhanced with KL-6 serum level for predicting disease progression in interstitial lung diseases (ILD)
Authors: F. Bonella, B. Hegedues, E. Gottstein, C. Taube / Conference Paper/ Sept. 2020
Inhaled Molgramostim Therapy in Autoimmune Pulmonary Alveolar Proteinosis
Authors: B.C. Trapnell, Y. Inoue, F. Bonella, T. Jouhikainen /Sept. 2020 New England Journal of Medicine 383(17) DOI:10.1056/NEJMoa1913590
Ganzlungenlavage bei pulmonaler Alveolarproteinose – Schritt für Schritt
Authors: J. Wälscher, T.E.Wessendorf, M.Rocha, F. Bonella // Oct. 2020, Pneumologie 74(10):660-664 DOI:10.1055/a-0978-9137
Inhaled Molgramostim Therapy in Autoimmune Pulmonary Alveolar Proteinosis (As a collaborator)
Authors: B.C. Trapnell, Y. Inoue, F. Bonella, T. Wang / Dec. 2020,New England Journal of Medicine 383(17):1635-1644
Inhaled Molgramostim Therapy in Autoimmune Pulmonary Alveolar Proteinosis (As a colaborator)
Authors: B.C. Trapnell, Y.Inoue, F. Bonella, T. Wang / New England Journal of Medicine 383(17):1635-1644, / Dec. 2020
Potential clinical utility of MUC5B und TOLLIP single nucleotide polymorphisms (SNPs) in the management of patients with IPF
Authors: F. Bonella, I.Campo, M. Zorzetto, U. Costabel / Dec. 2020 PrePrint; DOI:10.21203/rs.3.rs-133655/v2
Potential Clinical Utility of MUC5B und TOLLIP Single Nucleotide Polymorphisms (SNPs) in the Management of Patients with IPF
Authors: F. Bonella, I. Campo, M. Zorzetto, U. Costabel / Dec .2020 PrePrint ; DOI:10.21203/rs.3.rs-133655/v1
2019
Fatigue Assessment Scale Supplement manuscript
Authors: Mareye Voortman, C.M.R.Hendriks, Marjon Elfferich, Francesco Bonella, Janne Moller, Jolanda De Vries, Ulrich Costabel, M. Drent / Conference Paper, /LUNG-D-18-00439, January 2019
Mortalité prédite versus observée dans les essais cliniques du nintédanib dans la fibrose pulmonaire idiopathique (FPI)
Authors: Marlies Wijsenbeek, Francesco Bonella. January 2019
2022
The ERN-LUNG Population Registry: Aims, Software-Implementation and First Results.
Authors: Jannik Schaaf, Axel Zieschank, Jens Goebel, Thomas Otto Friedrich Wagner, Petra Pennekamp, Heymut Omran, Holger Storf. Stud Health Technol Inform. 2022 Jun 29;295:55-58. doi: 10.3233/SHTI220659
2021
Worldwide experiences and opinions of healthcare providers on eHealth for patients with interstitial lung diseases in the COVID-19 era
Authors: Gizal Nakshbandi, Catharina C. Moor, Kerri A. Johannson, Toby M. Maher, Michael Kreuter, Marlies S. Wijsenbeek. ERJ Open Res, 7 (3) 00405-2021; 10.1183/23120541.00405-2021
Authors: Jannik Schaaf, James Chalmers, Heymut Omran, Petra Pennekamp, Olivier Sitbon, Thomas OF Wagner, Abilio Reis, Dennis Kadioglu, Holger Storf. Stud Health Technol Inform. 2021 May 24;278:41-48. doi: 10.3233/SHTI210049.
[se-atlas.de-Medical care atlas for people with rare diseases]
Authors:
Michaela Neff, Jannik Schaaf, Niels Tegtbauer, Johanna Schäfer, Manuela Till, Thomas O F Wagner, Holm Graeßner, Christine Mundlos, Holger Storf Internist (Berl). 2021 Oct;62(10):1115-1122. doi: 10.1007/s00108-021-01085-y. Epub 2021 Jul 20.
How to design a registry for undiagnosed patients in the framework of rare disease diagnosis: suggestions on software, data set and coding system.
Authors: Berger A , Rustemeier AK, Göbel J, Kadioglu D, Britz V, Schubert K, Mohnike K, Storf H, Thomas O. F. Wagner. Orphanet J Rare Dis (2021) 16:198 https://doi.org/10.1186/s13023-021-01831-3
2020
The Status Quo of Rare Diseases Centres for the Development of a Clinical Decision Support System – A Cross-Sectional Study.
Authors: Schaaf J, Sedlmayr M, Prokosch HU, Ganslandt T, Schade-Brittinger C, von Wagner M, Kadioglu D, Schubert K, Lee-Kirsch MA, Kraemer BK, Winner B, Mueller T, Schaefer JR, Wagner TOF, Bruckner-Tuderman L, Tuescher O, Boeker M, Storf H
Patient registries for rare diseases in Germany: concept paper of the NAMSE strategy group [Article in German]
Authors: Storf H, Stausberg J, Kindle G, Quadder B, Schlangen M, Walter MC, Ückert F, Wagner TOF; NAMSE-Register-StrategiegruppeImplementation of EXABO: an Expert Advisory Board for the European Reference Network for Rare Respiratory Diseases.
Authors: Walther D, Steinmann O, Wagner TOF, Storf H
2019
Finding the Needle in the Hay Stack: An Open Architecture to Support Diagnosis of Undiagnosed Patients.
Authors: Schaaf J, Boeker M, Ganslandt T, Haverkamp C, Hermann T, Kadioglu D, Prokosch HU, Wagner TOF, von Wagner M, Schaefer J, Sedlmayr M, Storf H
2018
Conception of an Expert Advisory Board for the European Reference Network for Rare Respiratory Diseases.
Authors: Walther D, Steinmann O, Schaefer J, Wagner TOF, Storf H
2021
Educational aspects of rare and orphan lung diseases (Review)
Authors: Tiago M. Alfaro, Marlies S. Wijsenbeek, Pippa Powell, Daiana Stolz, John R. Hurst, Michael Kreuter and Catharina C. Moor). Respir Res. 2021 Mar 24;22(1):92. doi: 10.1186/s12931-021-01676-1
2019
Where are the gaps in education in the field of rare lung disease? Perspectives from the ERN-LUNG educational programme survey.
Authors: Powell P, Kreuter M, Wijsenbeek-Lourens M.
2025
Access to Lymphangioleiomyomatosis Care Across Europe: An ERN Survey of Clinical Practice and the Case for a European Registry and Trial Network.
Authors: Donal O’Malley, Elisabeth Humbert-Dorfmüller , Joan A Barberà , Aernoud van Batenburg, Elisabeth Bendstrup, Vincent Cottin, Davide Elia , Antoine Froidure , Jan Grutters , Sergio Harari , Fernanda Hernandez-Gonzalez, Maria Kokosi, Thomas Kromann Lund, Effrosyni D. Manali , Gergely Meszaros , Geert van Pottelberge , Elzbieta Radzikowska, Jacobo Sellares , Anna Svenningsson , Marcel Veltkamp , Thomas Wagner , Helge Hebestreit , Cormac McCarthy. ERJ Open Res. 07/2025. doi: https://doi.org/10.1183/23120541.01010-2025.
Modulation of infiltrating CD206-positive macrophages restricts progression of pulmonary lymphangioleiomyomatosis (LAM)
Authors: Heng-Jia Liu, Remi Diesler, Joelle Chami, Boxiang Wu, Heng Du, Yue Jin, Melissa Daou, Nicola Alesi, Damir Khabibullin, Caroline Leroux, Vincent Cottin, Lloyd G Cantley, Udo Rudloff, Elizabeth P Henske
2021
Looking forward: key initiatives to improve the care of rare diseases and streamline the delivery of medicines and vaccines in Europe.
Authors: Marc Humbert, Anita K Simonds. Am J Physiol Lung Cell Mol Physiol. 2021 Sep 1;321(3):L616-L618.doi: 10.1152/ajplung.00317.2021. Epub 2021 Jul 28.
2020
COVID-19 in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: a reference centre survey.
Authors: Belge C, Quarck R, Godinas L, Montani D, Escribano Subias P, Vachiéry JL, Nashat H, Pepke-Zaba J, Humbert M, Delcroix M.
Serum KL-6 concentrations as a novel biomarker of severe COVID-19.
Authors: d’Alessandro M, Cameli P, Refini RM, Bergantini L, Alonzi V, Lanzarone N, Bennett D, Rana GD, Montagnani F, Scolletta S, Franchi F, Frediani B, Valente S, Mazzei MA, Bonella F, Bargagli E.
Impact of COVID-19 on people with cystic fibrosis.
Authors: Colombo C, Burgel PR, Gartner S, van Koningsbruggen-Rietschel S, Naehrlich L, Sermet-Gaudelus I, Southern KW.
2025
Authors: Jens Gottlieb, Vos R, Jaksch P, Hellemons M, Holm AM, Morlacchi LC, Magnusson J, Alonso Moralejo R, Mora-Cuesta VM, Ennekes V, Reed A, Merveilleux Du Vignaux C, Hettich I, Bennett D, Hecker M, Wald A, Guk S, Skride A, Nolde A, Knoop C, Meloni F,, Tikkanen J, Larsson H, Tissot A, Riddell P, Le Pavec J, Perch M, Renaud-Picard B, Carlier FM, Müller V, Parmar J, Havlin J, Laporta R, Schuurmans MM, Harlander M, Zenglen S, Brugiere O, Kneidinger N, Fisher A, Saez-Gimenez B. ERJ Open Res. 05/2025. doi: 10.1183/23120541.00675-2024. PMID: 40470157 PMCID: PMC12134928
Authors: Rodríguez-Chiaradía DA, Torres-Castro R, Piccari L, García-Ortega A, Perez-Peñate GM, de Miguel-Díez J, Perez-Rojo R, Cano-Pumarega I, Mora-Cuesta V, Blanco I, Figueira-Gonçalves JM, Del Pozo R, López-Meseguer M, Martínez-Meñaca A, Tenes A, Molina-Molina M, Barberà JA. Arch Bronconeumol. 03/2025. doi: 10.1016/j.arbres.2025.03.015. PMID: 40222881
Authors: Mora-Cuesta VM, Martínez-Meñaca A, Iturbe-Fernández D, Tello-Mena S, Izquierdo-Cuervo S, García-Camarero T, Gil-Ongay A, Alonso-Lecue P, Cifrián-Martínez JM, Rodríguez-Chiaradia DA, Escribano-Subías P, Barreiro E. Pulm Circ. 06/2025. doi: 10.1002/pul2.70107. PMID: 40463492 PMCID: 12130283
Authors: Mora-Cuesta VM, Murillo-Brito DA, Argos-Vélez PJ, Iturbe-Fernández D, Naranjo-Gozalo S, Sánchez-Moreno L, Tello-Mena S, Berjón-de la Vega L, Izquierdo-Cuervo S, Poo-Fernández C, Andia-Torrico D, Alonso-Lecue P, Ballesteros-Sanz MA, Mons-Lera RJ, Cifrián-Martínez JM. Heart Lung. 11/2025. doi: 10.1016/j.hrtlng.2025.07.010. PMID: 40682990
Authors: Poo-Fernández C, Iturbe-Fernández D, Tello-Mena S, Izquierdo-Cuervo S, Sánchez-Moreno L, Murillo-Brito DA, Naranjo-Gozalo S, Mons-Lera R, Alonso-Lecue P, Cifrián-Martínez JM, Martínez-Meñaca A, Mora-Cuesta VM. J Thorac Dis. 07/2025. doi: 10.21037/jtd-24-1841. PMID: 40809276 PMCID: 12340290
From resistance to response: Bacteriophages in multidrug-resistant Mycobacterium abscessus after lung transplantation.
Authors: Ferreiro-Posse A. Am J Transplant. 11/2025. doi: 10.1016/j.ajt.2025.11.008.
A 2-year Review of the Diagnostic Performance of Serum and Bronchoalveolar Lavage Galactomannan Testing in Lung Transplant Recipients in a National Heart and Lung Transplant Centre.
Authors: O’Donnell C, Lynch B, O’Sullivan L, Killarney A, Murray M, Riddell P, Hannan MM. Transpl Infect Dis. 27/2025. doi: 10.1111/tid.14404.
Pathologists’ experience and routine practice in high-volume lung transplant centers: An international survey.
Authors: Calabrese F, Hwang DM, Lunardi F, Iannaco R, Adam J, Akyürek LM, Adam B, Jan B, Berry G, D’Sa L, Fabre A, Fattori A, Fishbein GA, Fredenburg KM, Romero TH, Husain AN, Jones K, Kern I, Lin CY, Louis R, … Levine DJ. JHLT Open. 11/2025. doi: 10.1016/j.jhlto.2025.100415.
Post-Lung Transplantation Care: The Case for Transplant Pulmonologist-Led Coordination.
Authors: Gottlieb J. Chest. 12/2025. doi: 10.1016/j.chest.2025.06.053
2024
Chronic lung allograft dysfunction after lung transplantation – prevention, diagnosis and treatment in 44 European centers.
Authors: Gottlieb J, Vos R, Jaksch P, Hellemons M, Holm AM, Morlacchi LC, Magnusson J, Alonso Moralejo R, Mora-Cuesta VM, Ennekes V, Reed A, Merveilleux C, Vignaux D, Hettich I, Bennett D, Hecker M, Wald A, Guk S, Skride A, Nolde A, Knoop C, Meloni F, Tikkanen J, Larsson H, Tissot A, Riddell P, Le Pavec J, Perch M, Renaud-Picard B, Carlier FM, Müller V, Parmar J, Havlin J, Laporta R, Schuurmans MM, Harlander M, Zenglen S, Brugiere O, Kneidinger N, Fisher A, Saez-Gimenez B. ERJ Open Research 2025 00675-2024; DOI: https://doi.org/10.1183/23120541.00675-2024
2023
Effect of once-per-day tacrolimus versus twice-per-day ciclosporin on 3-year incidence of chronic lung allograft dysfunction after lung
transplantation in Scandinavia (ScanCLAD): a multicentre randomised controlled trial.
Authors: Göran Dellgren, Gerdt Riise, Jesper Magnusson. Lancet 2023,
2022
Are anaplastic lymphoma kinase (ALK) and O6-methylguanine-DNA methyltransferase (MGMT) promoter methylation driver biomarkers of pulmonary neuroendocrine tumors (NETs) and carcinomas (NECs)?
Authors: Hiddinga B, Zwaenepoel K, Janssens A, Van Meerbeeck J, Pauwels P.Oncotarget. 2022 Jun 1;13:800-809. doi: 10.18632/oncotarget.28240. eCollection 2022.PMID: 35677534 Free PMC article.
Evaluation of Two Different CMV-Immunoglobulin Regimens for Combined CMV Prophylaxis in High-Risk Patients following Lung Transplant.
Authors: Víctor Manuel Mora Cuesta; Piedad Usstti Gil; Alicia De Pablo Gafas; David Iturbe Fernández; Rosalía Laporta Hernández; Rodrigo Alonso Moralejo; Myriam Aguilar Pérez; Carlos Andrés Quezada Loaiza; José Manuel Cifrián Martínez. MDPI, 22/12/2022. DOI: 10.3390/microorganisms11010032
Calcineurin inhibitors’ impact on cardiovascular and renal function, a descriptive study in lung transplant recipients from the North of Spain.
Authors: Rita Nogueiras Álvarez; Víctor Manuel Mora Cuesta; José Manuel Cifrián Martínez; María de los Ángeles Cos Cossío; María del Mar García Saiz. Scientific Reports. Nature Publishing Group, 12/12/2022. DOI: 10.1038/s41598-022-25445-2
Antifibrotics and lung transplantation: A Spanish multicentre case-controlled study.
Authors: Víctor Manuel Mora Cuesta; David Iturbe Fernández; Silvia Aguado Ibáñez; Gabriel Anguera De Francisco; Juan Margallo Iribarnegaray; Javier Carrillo Hernández Rubio; Juan Pablo Reig Mezquida; Virginia Pérez Luz; Rosalía Laporta Hernández; Alicia De Pablo Gafas; Amparo Solé Jover; José Manuel Cifrián Martínez. Respirology. 27, Wiley, 28/08/2022. DOI: 0.1111/resp.14352
Early onset of azithromycin to prevent CLAD in ling transplantation: promising results of a retrospective single centre experience.
Authors: Marta Cristeto Porras; Víctor Manuel Mora Cuesta; David Iturbe Fernández; Sandra Tello Mena; Pilar Alonso Lecue; Laura Sánchez Moreno; Eduardo Miñambres García; Sara Naranjo Gozalo; Sheila Izquierdo Cuervo; José Manuel Cifrián Martínez. Clinical Transplantation. Wiley, 26/10/2022. DOI: 10.1111/ctr.14832
Lung Rapid Recovery Procurement Combined with Abdominal Normothermic Regional Perfusion in Controlled Donation after Circulatory Death.
Authors: Sara Naranjo Gozalo; María de los Ángeles Ballesteros Sanz; Carlos Álvarez De Arriba; Víctor Manuel Mora Cuesta; Eduardo Miñambres García; Laura Sánchez Moreno. Journal of Visualized Experiments. 15/08/2022. DOI: 10.3791/63975
Myeloid-Derived Suppressor Cells Are Increased in Lung Transplant Recipients and Regulated by Immunosuppressive Therapy.
Authors: María Iglesias Escudero; David San Segundo Arribas; David Merino Fernández; Víctor Manuel Mora Cuesta; Patricia Lamadrid Perojo; Marta Alonso Peña; Sandra Raso Torres; David Iturbe Fernández; Sonia Fernández Rozas; José Manuel Cifrián Martínez; Marcos López Hoyos.. Frontiers in Inmunology. 10/01/2022. DOI: 10.3389/fimmu.2021.788851
Lung transplantation in HIV-positive patients: a european retrospective cohort study.
Authors: Rouzaud C, Berastegui C, Picard C, Vos R, Savale L, Demant X, Bertani A, Verschuuren E, Jaksch P, Reed A, Corinna Morlacchi L, Reynaud-Gaubert M, Lortholary O, Fadel E, Humbert M, Gottlieb J, Le Pavec J. Eur Respir J 2022; 60:2200189.