Universität zu Köln

Behr, Juergen, Prasse, Antje ORCID: 0000-0002-7336-7458, Wirtz, Hubert, Koschel, Dirk, Pittrow, David, Held, Matthias, Klotsche, Jens, Andreas, Stefan ORCID: 0000-0003-3918-6909, Claussen, Martin, Grohe, Christian, Wilkens, Henrike, Hagmeyer, Lars, Skowasch, Dirk, Meyer, Joachim F., Kirschner, Joachim, Glaeser, Sven, Kahn, Nicolas, Welte, Tobias, Neurohr, Claus, Schwaiblmair, Martin, Bahmer, Thomas, Oqueka, Tim, Frankenberger, Marion and Kreuter, Michael (2020). 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. Eur. Resp. J., 56 (2). SHEFFIELD: EUROPEAN RESPIRATORY SOC JOURNALS LTD. ISSN 1399-3003

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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 +/- so age was 69.8 +/- 9.1 years, and 81.0% were male. The mean +/- so 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 +/- so 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 su vests that in clinical practice, premature mortality of IPF patients eventually occurs despite stable measurements for FVC and D-LCO.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Behr, Juergen UNSPECIFIED UNSPECIFIED UNSPECIFIED Prasse, Antje UNSPECIFIED orcid.org/0000-0002-7336-7458 UNSPECIFIED Wirtz, Hubert UNSPECIFIED UNSPECIFIED UNSPECIFIED Koschel, Dirk UNSPECIFIED UNSPECIFIED UNSPECIFIED Pittrow, David UNSPECIFIED UNSPECIFIED UNSPECIFIED Held, Matthias UNSPECIFIED UNSPECIFIED UNSPECIFIED Klotsche, Jens UNSPECIFIED UNSPECIFIED UNSPECIFIED Andreas, Stefan UNSPECIFIED orcid.org/0000-0003-3918-6909 UNSPECIFIED Claussen, Martin UNSPECIFIED UNSPECIFIED UNSPECIFIED Grohe, Christian UNSPECIFIED UNSPECIFIED UNSPECIFIED Wilkens, Henrike UNSPECIFIED UNSPECIFIED UNSPECIFIED Hagmeyer, Lars UNSPECIFIED UNSPECIFIED UNSPECIFIED Skowasch, Dirk UNSPECIFIED UNSPECIFIED UNSPECIFIED Meyer, Joachim F. UNSPECIFIED UNSPECIFIED UNSPECIFIED Kirschner, Joachim UNSPECIFIED UNSPECIFIED UNSPECIFIED Glaeser, Sven UNSPECIFIED UNSPECIFIED UNSPECIFIED Kahn, Nicolas UNSPECIFIED UNSPECIFIED UNSPECIFIED Welte, Tobias UNSPECIFIED UNSPECIFIED UNSPECIFIED Neurohr, Claus UNSPECIFIED UNSPECIFIED UNSPECIFIED Schwaiblmair, Martin UNSPECIFIED UNSPECIFIED UNSPECIFIED Bahmer, Thomas UNSPECIFIED UNSPECIFIED UNSPECIFIED Oqueka, Tim UNSPECIFIED UNSPECIFIED UNSPECIFIED Frankenberger, Marion UNSPECIFIED UNSPECIFIED UNSPECIFIED Kreuter, Michael UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-324303
DOI:	10.1183/13993003.02279-2019
Journal or Publication Title:	Eur. Resp. J.
Volume:	56
Number:	2
Date:	2020
Publisher:	EUROPEAN RESPIRATORY SOC JOURNALS LTD
Place of Publication:	SHEFFIELD
ISSN:	1399-3003
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language CLINICAL-PRACTICE; DOUBLE-BLIND; PIRFENIDONE; ACETYLCYSTEINE; MANAGEMENT; THERAPY; SAFETY Multiple languages Respiratory System Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/32430