Extract
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 telomere-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 forced vital capacity (FVC) [5–7]. Two drugs, pirfenidone and nintedanib, have been shown to reduce the decline of FVC in IPF patients [8, 9]. So far, two studies have reported on the safety and effectiveness of pirfenidone in patients with a TRG mutation [6, 7] whereas no study has investigated nintedanib in this specific population. Thus, the aim of this retrospective study was to assess safety and efficacy of nintedanib and pirfenidone in IPF patients with a TRG mutation.
Abstract
This study suggests that pirfenidone and nintedanib can be used safely in IPF patients with a telomerase related gene mutation and that both drugs reduce FVC decline. These results should be confirmed in a larger prospective study. https://bit.ly/3k7b4Zx
Acknowledgement
We thank Camille Taille, Clairelyne Dupin (Bichat Hospital, Paris) and Julie Traclet (Louis Pradel Hospital, Lyon) for their efficient collaboration and for their help in collecting the data. V. Cottin is a member of ERN-LUNG.
Footnotes
In an exception to usual ERJ policy, a supplementary file containing a tabulated summary of the pathogenic telomerase-related gene mutations reported in this research letter is available from erj.ersjournals.com
Conflict of interest: A. Justet reports grants from Roche, personal fees from Boeringher Ingelheim, outside the submitted work.
Conflict of interest: D. Klay has nothing to disclose.
Conflict of interest: R. Porcher has nothing to disclose.
Conflict of interest: V. Cottin reports personal fees for advisory board work and lectures, and non-financial support for meeting attendance from Actelion, grants, personal fees for consultancy and lectures, and non-financial support for meeting attendance from Boehringer Ingelheim, personal fees for advisory board and data monitoring committee work from Bayer/MSD and Galapagos, personal fees for advisory board work and lectures from Novartis, personal fees for consultancy, lectures, steering committee and data monitoring committee work, and non-financial support for meeting attendance from Roche/Promedior, personal fees for lectures from Sanofi and AstraZeneca, personal fees for data monitoring committee work from Celgene and Galecto, personal fees for advisory board work from Shionogi, outside the submitted work.
Conflict of interest: K. Ahmad reports personal fees from Roche and Boeringher Ingelheim, outside the submitted work.
Conflict of interest: M. Molina-Molina reports grants and personal fees from Roche, Boehringer Ingelheim and Esteve-Teijin, personal fees from Chiesi, Pfizer and Galapagos, outside the submitted work.
Conflict of interest: H. Nunes reports personal fees from Intermune, Roche, Boehringer Ingelheim and Sanofi, outside the submitted work.
Conflict of interest: M. Reynaud-Gaubert has nothing to disclose.
Conflict of interest: J.M. Naccache has nothing to disclose.
Conflict of interest: E. Manali reports grants and personal fees from Roche and Boehringer Ingelheim, during the conduct of the study.
Conflict of interest: A. Froidure reports grants, personal fees and non-financial support from Roche and Boehringer Ingelheim, personal fees and non-financial support from AstraZeneca, personal fees from GlaxoSmithKline, outside the submitted work.
Conflict of interest: S. 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 and Savara-Serendex.
Conflict of interest: L. Wemeau has nothing to disclose.
Conflict of interest: C. Andrejak has nothing to disclose.
Conflict of interest: A. Gondouin has nothing to disclose.
Conflict of interest: S. Hirschi has nothing to disclose.
Conflict of interest: E. Blanchard has nothing to disclose.
Conflict of interest: B. Bondue reports grants and personal fees from Boeringher Ingleheim and Hoffman La Roche, outside the submitted work.
Conflict of interest: P. Bonniaud reports personal fees from Roche, Novartis, Boeringher, TEVA and AstraZeneca, outside the submitted work.
Conflict of interest: C. Tromeur has nothing to disclose.
Conflict of interest: G. Prevot reports personal fees from Actelion, Bayer, Boehringer Ingelheim and Roche, outside the submitted work.
Conflict of interest: S. Marchand-Adam has nothing to disclose.
Conflict of interest: M. Funke-Chambour reports grants from Roche and Boehringer Ingelheim, during the conduct of the study.
Conflict of interest: A.S. Gamez has nothing to disclose.
Conflict of interest: I. Ba has nothing to disclose.
Conflict of interest: S. Papiris reports grants and personal fees from Roche and Boehringer Ingelheim, during the conduct of the study.
Conflict of interest: J. Grutters has nothing to disclose.
Conflict of interest: B. Crestani reports personal fees from AstraZeneca and Sanofi, grants and personal fees from Boeringher Ingelheim and Roche, personal fees and non-financial support from BMS, outside the submitted work.
Conflict of interest: C. van Moorsel has nothing to disclose.
Conflict of interest: C. Kannengiesser has nothing to disclose.
Conflict of interest: R. Borie reports grants and personal fees from Boeringher Ingelheim and Roche, personal fees from Savapharma, outside the submitted work.
- Received July 4, 2020.
- Accepted September 9, 2020.
- Copyright ©ERS 2021
LIBRARY USERS
Log in through your institution
Purchase access
