Abstract
Rationale There is a paucity of observational data on antifibrotic therapy for idiopathic pulmonary fibrosis (IPF).
Objective We aimed to assess the course of disease of IPF patients with and without antifibrotic therapy under real-life conditions.
Methods We analysed data from a non-interventional, prospective cohort study of consecutively enrolled IPF patients from 20 ILD expert centres in Germany. Data quality was ensured by automated plausibility checks, on-site monitoring, and source data verification. Propensity scores were applied to account for known differences in baseline characteristics between patients with and without antifibrotic therapy.
Results Among the 588 patients suitable for analysis, the mean age was 69.8±9.1 years, and 81.0% were males. The mean duration of disease since diagnosis was 1.8±3.4 years. The mean % predicted value at baseline for forced vital capacity (FVC) and diffusion capacity (DLCO) were 68.6±18.8 and 37.8±18.5, respectively. During a mean follow-up of 1.2±0.7 years, 194 (33.0%) patients died. The one-year and two-year survival rates were 87% versus 46% and 62% versus 21%, respectively, for patients with versus without antifibrotic therapy. The risk of death was 37% lower in patients with antifibrotic therapy (HR=0.63, 95%CI: 0.45; 0.87; p=0.005). The results were robust (and remained statistically significant) on multivariable analysis. Overall decline of FVC and DLco was slow and did not differ significantly between patients with or without antifibrotic therapy.
Conclusions Survival was significantly higher in IPF patients with antifibrotic therapy, but the course of lung function parameters was similar in patients with and without antifibrotic therapy. This suggests that in clinical practice premature mortality of IPF patients eventually occurs despite stable measurements for FVC and DLco.
Footnotes
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Conflict of interest: Dr. Behr reports grants and personal fees from Boehriger Ingelheim, personal fees from Actelion, personal fees from Roche, personal fees from Galapagos, personal fees from Promedior, personal fees from BMS, personal fees from MSD, during the conduct of the study.
Conflict of interest: Dr. Prasse reports grants and personal fees from Roche/InterMune, grants and personal fees from Boehringer Ingelheim, outside the submitted work.
Conflict of interest: Dr. Wirtz reports personal fees from Roche, personal fees from Boehringer Ingelheim, outside the submitted work.
Conflict of interest: Dr. Koschel has nothing to disclose.
Conflict of interest: Dr. Pittrow reports personal fees from Actelion, personal fees from Bayer, personal fees from Boehringer Ingelheim, personal fees from Sanofi, personal fees from Biogen, personal fees from Shield, personal fees from MSD, outside the submitted work.
Conflict of interest: Dr. Held has nothing to disclose.
Conflict of interest: Dr. Klotsche has nothing to disclose.
Conflict of interest: Dr. Andreas reports grants and personal fees from Boehringer Ingelheim, personal fees from Roche, outside the submitted work.
Conflict of interest: Dr. Claussen reports personal fees from Roche, personal fees from Boehringer Ingelheim, outside the submitted work.
Conflict of interest: Dr. Grohé has nothing to disclose.
Conflict of interest: Dr. Wilkens reports personal fees from Boehringer Ingelheim, personal fees from Roche, personal fees from Actelion, personal fees from Biotest, personal fees from GSK, personal fees from Pfizer, personal fees from Bayer, outside the submitted work.
Conflict of interest: Dr. Hagmeier has nothing to disclose.
Conflict of interest: Dr. Skowasch reports personal fees from Boehringer Ingelheim, personal fees from Roche, outside the submitted work.
Conflict of interest: Dr. Meyer reports personal fees from Boehringer Ingelheim, personal fees from Novartis, outside the submitted work.
Conflict of interest: Dr. Kirschner has nothing to disclose.
Conflict of interest: Dr. Gläser reports grants and personal fees from Boehringer Ingelheim, personal fees from Roche, personal fees from Actelion, grants and personal fees from Novartis, personal fees from Berlin Chemie, personal fees from Astra, outside the submitted work.
Conflict of interest: Dr. Kahn has nothing to disclose.
Conflict of interest: Dr. Welte reports grants and personal fees from Boehringer Ingelheim, grants from Roche, outside the submitted work.
Conflict of interest: Dr. Neurohr reports personal fees from Boehringer Ingelheim, personal fees from Roche, outside the submitted work.
Conflict of interest: Dr. Schwaiblmair has nothing to disclose.
Conflict of interest: Dr. Bahmer reports grants from German Center of Lung Research (DZL), personal fees from Roche, personal fees from AstraZeneca, personal fees from Chiesi, personal fees from GSK, personal fees from Novartis, outside the submitted work.
Conflict of interest: Dr. Oqueka has nothing to disclose.
Conflict of interest: Dr. Frankenberger has nothing to disclose.
Conflict of interest: Dr. Kreuter reports grants and personal fees from Roche/InterMune, grants and personal fees from Boehringer Ingelheim, outside the submitted work.
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- Received November 26, 2019.
- Accepted April 7, 2020.
- Copyright ©ERS 2020