Survival and course of lung function in the presence or absence of antifibrotic treatment in patients with idiopathic pulmonary fibrosis: long-term results of the INSIGHTS-IPF registry

Abstract

Objective: There is a paucity of observational data on antifibrotic therapy for idiopathic pulmonary fibrosis (IPF). 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 interstitial lung disease 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±sd age was 69.8±9.1 years, and 81.0% were male. The mean±sd duration of disease since diagnosis was 1.8±3.4 years. The mean±sd value at baseline for forced vital capacity (FVC) and diffusion capacity (D LCO) were 68.6±18.8% predicted and 37.8±18.5% predicted, respectively. During a mean±sd follow-up of 1.2±0.7 years, 194 (33.0%) patients died. The 1-year and 2-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 (hazard ratio 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 D LCO 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 D LCO.

Conflict of interest statement

Conflict of interest: J. Behr reports grants and personal fees from Boehriger Ingelheim, personal fees from Actelion, Roche, Galapagos, Promedior, BMS and MSD, during the conduct of the study. Conflict of interest: A. Prasse reports grants and personal fees from Roche/InterMune and Boehringer Ingelheim, outside the submitted work. Conflict of interest: H. Wirtz reports personal fees from Roche and Boehringer Ingelheim, outside the submitted work. Conflict of interest: D. Koschel has nothing to disclose. Conflict of interest: D. Pittrow reports personal fees from Actelion, Bayer, Boehringer Ingelheim, Sanofi, Biogen, Shield and MSD, outside the submitted work. Conflict of interest: M. Held has nothing to disclose. Conflict of interest: J. Klotsche has nothing to disclose. Conflict of interest: S. Andreas reports grants and personal fees from Boehringer Ingelheim, personal fees from Roche, outside the submitted work. Conflict of interest: M. Claussen reports personal fees from Roche and Boehringer Ingelheim, outside the submitted work. Conflict of interest: C. Grohé has nothing to disclose. Conflict of interest: H. Wilkens reports personal fees from Boehringer Ingelheim, Roche, Actelion, Biotest, GSK, Pfizer and Bayer, outside the submitted work. Conflict of interest: L. Hagmeyer has nothing to disclose. Conflict of interest: D. Skowasch reports personal fees from Boehringer Ingelheim and Roche, outside the submitted work. Conflict of interest: J.F. Meyer reports personal fees for lectures from Boehringer Ingelheim and Novartis, outside the submitted work. Conflict of interest: J. Kirschner has nothing to disclose. Conflict of interest: S. Gläser reports grants and personal fees from Boehringer Ingelheim and Novartis, personal fees from Roche, Actelion, Berlin Chemie and Astra, outside the submitted work. Conflict of interest: N. Kahn has nothing to disclose. Conflict of interest: T. Welte reports grants and personal fees from Boehringer Ingelheim, grants from Roche, outside the submitted work. Conflict of interest: C. Neurohr reports personal fees from Boehringer Ingelheim and Roche, outside the submitted work. Conflict of interest: M. Schwaiblmair has nothing to disclose. Conflict of interest: T. Bahmer reports grants from German Center of Lung Research (DZL), personal fees from Roche, AstraZeneca, Chiesi, GSK and Novartis, outside the submitted work. Conflict of interest: T. Oqueka has nothing to disclose. Conflict of interest: M. Frankenberger has nothing to disclose. Conflict of interest: M. Kreuter reports grants and personal fees from Roche/InterMune and Boehringer Ingelheim, outside the submitted work.

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