Safety and efficacy of pirfenidone and nintedanib in patients with Idiopathic Pulmonary Fibrosis and carrying a telomere related gene mutation.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and deadly interstitial lung disease (ILD). Over the past decade, familial occurrence of IPF led to the identification of genetic susceptibility traits [1]. Germline pathogenic variations in telomeres related genes (TRG) such as TERT , TERC TINF2 , DKC1 , RTEL1 , PARN , NAF1 , ZCCHC8 , NHP2 , and NOP10 have been detected in 20–30% of patients with familial pulmonary fibrosis (FPF) and in 1–5% of sporadic IPF [2–4]. In comparison with IPF patients, carriers of a TRG mutation are significantly younger and show an accelerated decline of FVC [5–7]. Footnotes This manuscript has recently been accepted for publication in the European Respiratory Journal . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article. Conflict of interest: Dr. Justet reports grants from Roche, personal fees from Boeringher Ingelheim, outside the submitted work;. Conflict of interest: Dr. Klay has nothing to disclose. Conflict of interest: Dr. Porcher has nothing to disclose. Conflict of interest: Dr. Cottin reports personal fees and non-financial support from Actelion, grants, personal fees and non-financial support from Boehringer Ingelheim, personal fees from Bayer / MSD, personal fees from Novartis, personal fees and non-financial support from Roche / Promedior, personal fees from Sanofi, personal fees from Celgene, personal fees from Galapagos, personal fees from Galecto, personal fees from Shionogi, personal fees from Astra Zeneca, outside the submitted work;. Conflict of interest: Dr. Ahmad reports personal fees from Roche, personal fees from Boeringher Ingelheim, outside the submitted work;. Conflict of interest: Dr. MOLINA-MOLINA reports grants and personal fees from Roche, grants and personal fees from Boehringer Ing, personal fees from Chiesi, personal fees from Pfizer , grants and personal fees from Esteve-Teijin, personal fees from Galapagos, outside the submitted work;. Conflict of interest: Dr. Nunes reports personal fees from Intermune, personal fees from Roche, personal fees from Boehringer-Ingelheim, personal fees from Sanofi, outside the submitted work;. Conflict of interest: Dr. Reynaud-Gaubert has nothing to disclose. Conflict of interest: Dr. Naccache has nothing to disclose. Conflict of interest: Dr. MANALI reports grants and personal fees from ROCHE , grants and personal fees from BOEHRINGER INGELHEIM , during the conduct of the study;. Conflict of interest: Dr. FROIDURE reports grants, personal fees and non-financial support from Roche, grants, personal fees and non-financial support from Boehringer Ingelheim, personal fees and non-financial support from AstraZeneca, personal fees from GlaxoSmithKline, outside the submitted work;. Conflict of interest: Dr. Jouneau reports fees, funding or reimbursement for national and international conferences, boards, expert or opinion groups, research projects over the past 3 years from AIRB, Bellorophon Therapeutics, Biogen, Boehringer, Chiesi, Fibrogen, Galecto Biotech, Genzyme, Gilead, LVL, Novartis, Olam Pharm, Pfizer, Pliant Therapeutics, Roche, Sanofi, Savara-Serendex.. Conflict of interest: Dr. Wemeau has nothing to disclose. Conflict of interest: Dr. Andrejak has nothing to disclose. Conflict of interest: Dr. Gondouin has nothing to disclose. Conflict of interest: Dr. Hirschi has nothing to disclose. Conflict of interest: Dr. Blanchard has nothing to disclose. Conflict of interest: Dr. Bondue reports grants and personal fees from Boeringher Ingleheim, grants and personal fees from Hoffman La Roche , outside the submitted work;. Conflict of interest: Dr. Bonniaud reports personal fees from Roche, personal fees from Novartis, personal fees from Boeringher, personal fees from TEVA, from Astra Zeneca, outside the submitted work;. Conflict of interest: Dr. Tromeur has nothing to disclose. Conflict of interest: Dr. Prevot reports personal fees from Actelion, personal fees from Bayer, personal fees from Boehringer Ingelheim, personal fees from Roche , outside the submitted work;. Conflict of interest: Dr. Marchand Adam has nothing to disclose. Conflict of interest: Dr. Funke-Chambour reports grants from Roche, grants from Boehringer Ingelheim, during the conduct of the study;. Conflict of interest: Dr. Gamez has nothing to disclose. Conflict of interest: Dr. Ba has nothing to disclose. Conflict of interest: Dr. PAPIRIS reports grants and personal fees from ROCHE , grants and personal fees from BOEHRINGER INGELHEIM , during the conduct of the study;. Conflict of interest: Dr. Grutters has nothing to disclose. Conflict of interest: Dr. Crestani reports personal fees from Astra Zeneca, personal fees from Sanofi, grants and personal fees from Boeringher Ingelheim, grants and personal fees from Roche, personal fees and non-financial support from BMS, outside the submitted work;. Conflict of interest: Dr. van Moorsel has nothing to disclose. Conflict of interest: Dr. Kannengiesser has nothing to disclose. Conflict of interest: Dr. Borie reports grants and personal fees from Boeringher Ingelheim, grants and personal fees from Roche, personal fees from Savapharma, outside the submitted work;.