Safety and efficacy of pirfenidone in patients carrying telomerase complex mutation

Aurélien Justet; Gabriel Thabut; Effrosyni Manali; Maria Molina Molina; Caroline Kannengiesser; Jacques Cadranel; Vincent Cottin; Anne Gondouin; Hilario Nunes; Eline Magois; Cécile Tromeur; Grégoire Prevot; Spyros Papiris; Sylvain Marchand-Adam; Anne Sophie Gamez; Martine Reynaud-Gaubert; Lidwine Wemeau; Bruno Crestani; Raphael Borie

doi:10.1183/13993003.01875-2017

Safety and efficacy of pirfenidone in patients carrying telomerase complex mutation

Eur Respir J. 2018 Mar 15;51(3):1701875. doi: 10.1183/13993003.01875-2017. Print 2018 Mar.

Authors

Aurélien Justet^{1

2}, Gabriel Thabut^{2

3}, Effrosyni Manali⁴, Maria Molina Molina⁵, Caroline Kannengiesser⁶, Jacques Cadranel⁷, Vincent Cottin⁸, Anne Gondouin⁹, Hilario Nunes¹⁰, Eline Magois¹¹, Cécile Tromeur¹², Grégoire Prevot¹³, Spyros Papiris⁴, Sylvain Marchand-Adam¹⁴, Anne Sophie Gamez¹⁵, Martine Reynaud-Gaubert¹⁶, Lidwine Wemeau¹⁷, Bruno Crestani^{1

2}, Raphael Borie^{18

2}

Affiliations

¹ APHP, Hôpital Bichat, Service de Pneumologie A, DHU FIRE, Paris, France.
² INSERM, Unité 1152, Université Paris Diderot, Paris, France.
³ APHP, Hôpital Bichat, Service de Pneumologie B, Paris, France.
⁴ Respiratory Medicine Dept, 'Attikon' University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Athens, Greece.
⁵ Unit of Interstitial Lung Diseases, Dept of Pneumology, University Hospital of Bellvitge, Barcelona, Spain.
⁶ APHP, Hôpital Bichat, Département de Génétique, Université Paris Diderot, Paris, France.
⁷ APHP, Service de Pneumologie, maladies pulmonaires rares, Hôpital Tenon, Paris, France.
⁸ Service de Pneumologie, Hôpital Louis Pradel, Université Claude Bernard Lyon 1, Lyon, France.
⁹ CHU de Besançon, Service de Pneumologie, Besançon, France.
¹⁰ APHP, Service de Pneumologie, maladies pulmonaires rares, Hôpital Avicenne, Bobigny, France.
¹¹ Service de Pneumologie, Hôpital d'Amiens, Université de Picardie Jules Verne, Amiens, France.
¹² Université Européenne de Bretagne, UBO, EA3878 (GETBO) IFR 148, CHU de la Cavale Blanche, Département de médecine interne et de pneumologie, Brest, France.
¹³ Service de Pneumologie, Hôpital Larrey, Toulouse, France.
¹⁴ CHU de Tours, Service de Pneumologie et Explorations Fonctionnelles Respiratoires, Tours, France.
¹⁵ Département de Pneumologie et Addictologie, Hôpital Arnaud de Villeneuve, CHU Montpellier, Montpellier, France.
¹⁶ Service de Pneumologie, Hôpital Nord, Marseille, France.
¹⁷ Service de Pneumologie, CHRU de Lille, Lille, France.
¹⁸ APHP, Hôpital Bichat, Service de Pneumologie A, DHU FIRE, Paris, France [email protected].

PMID: 29449422
DOI: 10.1183/13993003.01875-2017

No abstract available

Publication types

Letter
Multicenter Study
Research Support, Non-U.S. Gov't

MeSH terms

Aged
Anti-Inflammatory Agents, Non-Steroidal / adverse effects
Anti-Inflammatory Agents, Non-Steroidal / therapeutic use*
Female
France
Greece
Heterozygote
Humans
Male
Middle Aged
Mutation*
Patient Safety
Pulmonary Fibrosis / genetics*
Pyridones / adverse effects
Pyridones / therapeutic use*
RNA / genetics
Retrospective Studies
Spain
Telomerase / genetics*

Substances

Anti-Inflammatory Agents, Non-Steroidal
Pyridones
telomerase RNA
RNA
pirfenidone
TERT protein, human
Telomerase