OrphaLung
Publications OrphaLung
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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. CT features of interstitial lung disease in systemic lupus erythematosus and overlap lupus-other connective tissue disease. Eur Radiol. 2026 May;36(5):4074-4085. doi:10.1007/s00330-025-12106-x. Epub 2025 Nov 19. PMID: 41261180
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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. Efficacy and safety of danazol for pulmonary fibrosis or bone-marrow failure associated with telomere-related gene mutation. ERJ Open Res. 2026 Apr 7;12(2):00905-2025. doi: 10.1183/23120541.00905-2025. PMID: 41953763; PMCID: PMC13054709
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Diesler R, Gallay L, Uzunhan Y, Borie R, Reynaud-Gaubert M, Valentin V, Ahmad K, Lazor R, Wemeau L, Traclet J, Rigaud P, Marchand-Adam S, Israël-Biet D, Hirschi S, Bonniaud P, Froidure A, Montani D, Naccache JM, Romain-Scelle N, Cottin V; OrphaLung Network. Risk of hospitalisation and mortality among patients with interstitial lung disease and COVID-19: A French multicentre prospective cohort. Pulmonology. 2025 Dec 31;31(1):2598693. doi:10.1080/25310429.2025.2598693. Epub 2025 Dec 9. PMID: 41363029
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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. Nintedanib Combined With Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis or Progressive Pulmonary Fibrosis: A Long-Term Retrospective Multicentre Study (Combi-PF). Arch Bronconeumol. 2025 Oct 10:S0300-2896(25)00355-2. English, Spanish. doi: 10.1016/j.arbres.2025.10.004. Epub ahead of print. PMID: 41152077
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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. Molecular Investigation in Early-Onset Interstitial Lung Disease: Results From 699 Unrelated Patients. Respirology. 2025 Oct 3. doi: 10.1111/resp.70132. Epub ahead of print. PMID: 41041870
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Ratsihorimanana R, Maitre T, Dusselier M, Triet Ngo M, Mangiapan G, Fournier C, Bourdin A, Jouneau S, Matar M, Favrolt N, Egenod T, Michel Vergnon J, Wermert D, François Boitiaux J, Borie R, Caliandro R, Freynet O, Gounant V, Mankikian J, Camuset J, Elabbadi A, Parrot A, Calvani J, Fortin M, Guibert N, Cottin V, Cadranel J.
Recurrent respiratory papillomatosis in adults with lower respiratory tract involvement: a retrospective study of the OrphaLung and GETIF networks.
Eur Respir J. 2025 Feb 6:2400618. doi: 10.1183/13993003.00618-2024. Online ahead of print. PMID: 39915056
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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. Pulmonary fibrosis in patients with autoimmune pulmonary alveolar proteinosis: a retrospective nationwide cohort study. ERJ Open Res. 2024 Dec 2;10(6):00314-2024. doi: 10.1183/23120541.00314-2024. eCollection 2024 Nov.PMID: 39624377
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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.. 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. Respir Med Res. 2024 Nov 28;87:101144. doi: 10.1016/j.resmer.2024.101144. Epub ahead of print. PMID: 39693827
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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. Rituximab and mycophenolate mofetil in interstitial lung disease (EVER-ILD): 1-year follow-up results of a randomised controlled trial. Eur Respir J. 2024 Sep 12;64(3):2401368. doi: 10.1183/13993003.01368-2024. PMID: 39231630
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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. High risk of lung cancer in surfactant-related gene variant carriers. Eur Respir J. 2024 May 2;63(5):2301809. doi: 10.1183/13993003.01809-2023. PMID: 38575158; PMCID: PMC11063619
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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. Phenotypes and outcome of diffuse pulmonary non-amyloid light chain deposition disease. Respir Res. 2024 Apr 10;25(1):159. doi: 10.1186/s12931-024-02798-y. PMID: 38600600; PMCID: PMC11005206.
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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. Similarities and differences of interstitial lung disease associated with pathogenic variants in SFTPC and ABCA3 in adults. Respirology. 2024 Apr;29(4):312-323. doi: 10.1111/resp.14667. Epub 2024 Feb 12. PMID: 38345107.
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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. Chronic interstitial lung disease associated with systemic lupus erythematosus: A multicentric study of 89 cases. Respirology. 2024 Aug;29(8):713-721. doi: 10.1111/resp.14703. Epub 2024 Mar 17.
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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, MarquetteCH, Quétant S, Nunes H, Chenivesse C, Crestani B, Guirriec Y, Monnier D, MénardC, Tattevin P, Cottin V, Luque Paz D, Jouneau S; OrphaLung Network. Infections in autoimmune pulmonary alveolar proteinosis: a large retrospective cohort. Thorax. 2023 Sep 27:thorax-2023-220040. doi: 10.1136/thorax-2023-220040. Epub ahead of print. PMID: 37758458
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French recommendations for the diagnosis and management of lymphangioleiomyomatosis.
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
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Nicolas A, Leroy S, Mouthon L, Uzunhan Y, Cottin V, Mekinian A, Queyrel V, Hachulla E, Gachet B, aunay D, Martis N; Groupe Orphalung, Groupe Francophone de Recherche sur la Sclérodermie. Systemic sclerosis associated interstitial lung disease: a survey of current practices in France.Ther Adv Musculoskelet Dis. 2023 May 9;15:1759720X231159712. doi: 10.1177/1759720X231159712. Collection 2023. PMID: 37187855 Free PMC article.
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Delcros Q, Taillé C, Vallée A, Brun AL, Chenivesse C, Couture P, Daboussi S, Guilleminault L, Juster F, Kahn JE, Lefèvre G, Liozon E, Magnan A, Meireles S, Moulis G, Puech C, Tcherakian C, Traclet J, Uzunhan Y, Cottin V, Groh M; CEREO, OrphaLung and CRISALIS networks. Targeting interleukin-5/5R for the treatment of idiopathic chronic eosinophilic pneumonia. J Allergy Clin Immunol Pract. 2022 Dec 26:S2213-2198(22)01335-6. doi: 10.1016/j.jaip.2022.12.022. Online ahead of print. PMID: 36581069
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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. [French practical guidelines for the diagnosis and management of IPF - 2021 update, short version]. Rev Mal Respir. 2022 Mar 14:S0761-8425(22)00026-2. doi: 10.1016/j.rmr.2022.01.005. Online ahead of print.
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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.
Interstitial lung diseases associated with mutations of poly(A)-specific ribonuclease: A multicentre retrospective study.Respirology. 2022 Jan 3. doi: 10.1111/resp.14195. Online ahead of print.
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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.
Determinants of survival after lung transplantation in telomerase-related gene mutation carriers: a retrospective cohort. Am J Transplant. 2022 Apr;22(4):1236-1244. doi: 10.1111/ajt.16893. Epub 2021 Dec 28.PMID: 34854205.
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Naccache JM, Jouneau S, Didier M, Borie R, Cachanado M, Bourdin A, Reynaud-Gaubert M, Bonniaud P, Israël-Biet D, Prévot G, Hirschi S, Lebargy F, Marchand-Adam S, Bautin N, Traclet J, Gomez E, Leroy S, Gagnadoux F, Rivière F, Bergot E, Gondouin A, Blanchard E, Parrot A, Blanc FX, Chabrol A, Dominique S, Gibelin A, Tazi A, Berard L, Brillet PY, Debray MP, Rousseau A, Kerjouan M, Freynet O, Dombret MC, Gamez AS, Nieves A, Beltramo G, Pastré J, Le Borgne-Krams A, Dégot T, Launois C, Plantier L, Wémeau-Stervinou L, Cadranel J, Chenivesse C, Valeyre D, Crestani B, Cottin V, Simon T, Nunes H; EXAFIP investigators and the OrphaLung network.Cyclophosphamide added to glucocorticoids in acute exacerbation of idiopathic pulmonary fibrosis (EXAFIP): a randomised, double-blind, placebo-controlled, phase 3 trial.Lancet Respir Med. 2021 Sep 7:S2213-2600(21)00354-4. doi: 10.1016/S2213-2600(21)00354-4. Online ahead of print.
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Cottin V, Gueguen S, Jouneau S, Nunes H, Crestani B, Bonniaud P, Wémeau-Stervinou L, Reynaud-Gaubert M, Israël-Biet D, Cadranel J, Marchand-Adam S, Quétant S, Hirschi S, Montani D, Gamez AS, Chevereau M, Dufaure-Garé I, Amselem S, Clement A; OrphaLung and RespiFil Networks.Impact of Gender on the Characteristics of Patients with Idiopathic Pulmonary Fibrosis Included in the RaDiCo-ILD Cohort. Respiration. 2021 Sep 2:1-12. doi: 10.1159/000518008. Online ahead of print.
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Justet A, Klay D, Porcher R, Cottin V, Ahmad K, Molina Molina M, Nunes H, Reynaud-Gaubert M, Naccache JM, Manali E, Froidure A, Jouneau S, Wemeau L, Andrejak C, Gondouin A, Hirschi S, Blanchard E, Bondue B, Bonniaud P, Tromeur C, Prévot G, Marchand-Adam S, Funke-Chambour M, Gamez AS, Ba I, Papiris S, Grutters J, Crestani B, van Moorsel C, Kannengiesser C, Borie R; OrphaLung Network. Safety and efficacy of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis and carrying a telomere-related gene mutation. Eur Respir J. 2021 Feb 11;57(2):2003198. doi: 10.1183/13993003.03198-2020.
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Ferreira M, Borie R, Crestani B, Rigaud P, Wemeau L, Israel-Biet D, Leroy S, Quétant S, Plantier L, Dalphin JC, Cottin V, Marchand-Adam S; of the OrphaLung network.Efficace and safety of rituximab in patients with chronic hypersensitivity pneumonitis (cHP): A retrospective, multicentric, observational study. Respir Med. 2020 Sep 9;172:106146. doi: 10.1016/j.rmed.2020.106146. Online ahead of print.
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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, and OrphaLung. Hemodynamic response to treatment and outcome in pulmonary hypertension associated with interstitial lung disease versus pulmonary arterial hypertension in systemic sclerosis. Arthritis Rheumatol. 2020 Sep 6. doi: 10.1002/art.41512. Epub ahead of print. PMID: 32892515.
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Bejan-Angoulvant T, Naccache JM, Caille A, Borie R, Nunes H, Ferreira M, Cadranel J, Crestani B, Cottin V, Marchand-Adam S, and OrphaLung. Evaluation of efficacy and safety of rituximab in combination with mycophenolate mofetil in patients with nonspecific interstitial pneumonia non-responding to a first-line immunosuppressive treatment (EVER-ILD): A double-blind placebo-controlled randomized trial. Respir Med Res. 2020 May 23;78:100770. doi: 10.1016/j.resmer.2020.100770. Epub ahead of print. PMID: 32777737.
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Daccord C, Cottin V, Prévot G, Uzunhan Y, Mornex JF, Bonniaud P, Borie R, Briault A, Collonge-Rame MA, Crestani B, Devouassoux G, Freynet O, Gondouin A, Hauss PA, Khouatra C, Leroy S, Marchand-Adam S, Marquette C, Montani D, Naccache JM, Nadeau G, Poulalhon N, Reynaud-Gaubert M, Salaun M, Wallaert B, Cordier JF, Faouzi M, Lazor R; OrphaLung network. Lung function in Birt-Hogg-Dubé syndrome: a retrospective analysis of 96 patients. Orphanet J Rare Dis. 2020 May 24;15(1):120. doi: 10.1186/s13023-020-01402-y. PMID: 32448321; PMCID: PMC7245949.
