Safety and efficacy of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis and carrying a telomere-related gene mutation
Aurélien Justet, Dymph Klay, Raphaël Porcher, Vincent Cottin, Kais Ahmad, Maria Molina Molina, Hilario Nunes, Martine Reynaud-Gaubert, Jean Marc Naccache, Effrosyni Manali, Antoine Froidure, Stéphane Jouneau, Lidwine Wemeau, Claire Andrejak, Anne Gondouin, Sandrine Hirschi, Elodie Blanchard, Benjamin Bondue, Philippe Bonniaud, Cécile Tromeur, Grégoire Prévot, Sylvain Marchand-Adam, Manuela Funke-Chambour, Anne Sophie Gamez, Ibrahima Ba, Spyridon Papiris, Jan Grutters, Bruno Crestani, Coline van Moorsel, Caroline Kannengiesser, Raphaël Borie on behalf of the OrphaLung Network
European Respiratory Journal 2021 57: 2003198; DOI: 10.1183/13993003.03198-2020
Aurélien Justet
1Université de Paris, Reference center for rare pulmonary diseases, Service de Pneumologie A, Bichat Hospital, DHU APOLLO, APHP - Paris (France) - INSERM UMR 1152, Paris, France
2OrphaLung Network, Paris, France
3Center for rare pulmonary disease, Service de Pneumologie, CHU de Caen - ISTCT, UMR6030-CNRS-CEA-Université de Caen, Caen, France
Dymph Klay
4Interstitial Lung Diseases Center of Excellence, Dept of Pulmonology, St Antonius Hospital, Nieuwegein, The Netherlands
Raphaël Porcher
5Centre of Research in Epidemiology and Statistics Sorbonne Paris Cité -CRESS-UMR1153, Paris, France
Vincent Cottin
2OrphaLung Network, Paris, France
6National reference center for rare pulmonary diseases (OrphaLung), Dept of Respiratory Medicine, Louis Pradel Hospital; UMR754, Claude Bernard Lyon 1 University; Lyon, France
Kais Ahmad
2OrphaLung Network, Paris, France
6National reference center for rare pulmonary diseases (OrphaLung), Dept of Respiratory Medicine, Louis Pradel Hospital; UMR754, Claude Bernard Lyon 1 University; Lyon, France
Maria Molina Molina
7Unit of Interstitial Lung Diseases, Dept of Pneumology, University Hospital of Bellvitge, Barcelona, Spain
Hilario Nunes
2OrphaLung Network, Paris, France
8Reference center for rare pulmonary diseases APHP, Service de Pneumologie, Hôpital Avicenne, Bobigny, France
Martine Reynaud-Gaubert
2OrphaLung Network, Paris, France
9Center for rare pulmonary disease, Service de Pneumologie, Hôpital Nord, Marseille, France
Jean Marc Naccache
2OrphaLung Network, Paris, France
10Reference center for rare pulmonary diseases, APHP, Service de Pneumologie, Hôpital Tenon, Paris, France
Effrosyni Manali
11Respiratory Medicine Dept, ‘Attikon’ University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Athens, Greece
Antoine Froidure
12Cliniques Universitaires Saint-Luc, Service de Pneumologie, Bruxelles, France
Stéphane Jouneau
2OrphaLung Network, Paris, France
13Center for rare pulmonary disease, Centre Hospitalier Universitaire de Rennes, Service de Pneumologie, - IRSET (Institut de recherche en santé, environnement et travail) - UMR_S 1085, Université de Rennes 1, Rennes, France
Lidwine Wemeau
2OrphaLung Network, Paris, France
14Reference center for rare pulmonary diseases, Service de Pneumologie, CHRU de Lille, Lille, France
Claire Andrejak
2OrphaLung Network, Paris, France
15Center for rare pulmonary disease, Service de Pneumologie, Hôpital d'Amiens, Université de Picardie Jules Verne, Amiens, France
Anne Gondouin
2OrphaLung Network, Paris, France
16Center for rare pulmonary disease CHU de Besançon, Service de Pneumologie, Besançon, France
Sandrine Hirschi
2OrphaLung Network, Paris, France
17Center for rare pulmonary disease, Service de Pneumologie, Groupe de Transplantation Pulmonaire, Hôpitaux Universitaires de Strasbourg, Strasbourg, France
Elodie Blanchard
2OrphaLung Network, Paris, France
18Center for rare pulmonary disease, CHU de Bordeaux, Service de Pneumologie, Pessac, France
Benjamin Bondue
19Hôpital Erasme – ULB, Brussels, Belgium
Philippe Bonniaud
2OrphaLung Network, Paris, France
20Reference center for rare pulmonary diseases, Service de Pneumologie, Dijon, France
Cécile Tromeur
2OrphaLung Network, Paris, France
21CHU de la Cavale Blanche, Département de médecine interne et de pneumologie, Brest, France
Grégoire Prévot
2OrphaLung Network, Paris, France
22Center for rare pulmonary diseases, Service de Pneumologie, Hôpital Larrey, Toulouse, France
Sylvain Marchand-Adam
2OrphaLung Network, Paris, France
23Center for rare pulmonary diseases, CHU de Tours, Service de Pneumologie et Explorations Fonctionnelles Respiratoires, Tours, France
Manuela Funke-Chambour
24Service universitaire de pneumologie, Bern, Switzerland
Anne Sophie Gamez
2OrphaLung Network, Paris, France
25Center for rare pulmonary diseases, Département de Pneumologie et Addictologie, Hôpital Arnaud de Villeneuve, CHU Montpellier, Montpellier, France
Ibrahima Ba
2OrphaLung Network, Paris, France
26Dept of Genetics, APHP, Hôpital Bichat, Paris, France
Spyridon Papiris
11Respiratory Medicine Dept, ‘Attikon’ University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Athens, Greece
Jan Grutters
4Interstitial Lung Diseases Center of Excellence, Dept of Pulmonology, St Antonius Hospital, Nieuwegein, The Netherlands
Bruno Crestani
1Université de Paris, Reference center for rare pulmonary diseases, Service de Pneumologie A, Bichat Hospital, DHU APOLLO, APHP - Paris (France) - INSERM UMR 1152, Paris, France
2OrphaLung Network, Paris, France
Coline van Moorsel
4Interstitial Lung Diseases Center of Excellence, Dept of Pulmonology, St Antonius Hospital, Nieuwegein, The Netherlands
Caroline Kannengiesser
2OrphaLung Network, Paris, France
26Dept of Genetics, APHP, Hôpital Bichat, Paris, France
Raphaël Borie
1Université de Paris, Reference center for rare pulmonary diseases, Service de Pneumologie A, Bichat Hospital, DHU APOLLO, APHP - Paris (France) - INSERM UMR 1152, Paris, France
2OrphaLung Network, Paris, France
Figures
Supplementary Materials
Vol 57 Issue 2
Table of Contents
Safety and efficacy of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis and carrying a telomere-related gene mutation
Aurélien Justet, Dymph Klay, Raphaël Porcher, Vincent Cottin, Kais Ahmad, Maria Molina Molina, Hilario Nunes, Martine Reynaud-Gaubert, Jean Marc Naccache, Effrosyni Manali, Antoine Froidure, Stéphane Jouneau, Lidwine Wemeau, Claire Andrejak, Anne Gondouin, Sandrine Hirschi, Elodie Blanchard, Benjamin Bondue, Philippe Bonniaud, Cécile Tromeur, Grégoire Prévot, Sylvain Marchand-Adam, Manuela Funke-Chambour, Anne Sophie Gamez, Ibrahima Ba, Spyridon Papiris, Jan Grutters, Bruno Crestani, Coline van Moorsel, Caroline Kannengiesser, Raphaël Borie
European Respiratory Journal Feb 2021, 57 (2) 2003198; DOI: 10.1183/13993003.03198-2020
Safety and efficacy of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis and carrying a telomere-related gene mutation
Aurélien Justet, Dymph Klay, Raphaël Porcher, Vincent Cottin, Kais Ahmad, Maria Molina Molina, Hilario Nunes, Martine Reynaud-Gaubert, Jean Marc Naccache, Effrosyni Manali, Antoine Froidure, Stéphane Jouneau, Lidwine Wemeau, Claire Andrejak, Anne Gondouin, Sandrine Hirschi, Elodie Blanchard, Benjamin Bondue, Philippe Bonniaud, Cécile Tromeur, Grégoire Prévot, Sylvain Marchand-Adam, Manuela Funke-Chambour, Anne Sophie Gamez, Ibrahima Ba, Spyridon Papiris, Jan Grutters, Bruno Crestani, Coline van Moorsel, Caroline Kannengiesser, Raphaël Borie
European Respiratory Journal Feb 2021, 57 (2) 2003198; DOI: 10.1183/13993003.03198-2020