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

Full text not available from this repository.

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:
CreatorsEmailORCIDORCID Put Code
Behr, JuergenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Prasse, AntjeUNSPECIFIEDorcid.org/0000-0002-7336-7458UNSPECIFIED
Wirtz, HubertUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Koschel, DirkUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pittrow, DavidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Held, MatthiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Klotsche, JensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Andreas, StefanUNSPECIFIEDorcid.org/0000-0003-3918-6909UNSPECIFIED
Claussen, MartinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Grohe, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wilkens, HenrikeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hagmeyer, LarsUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Skowasch, DirkUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Meyer, Joachim F.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kirschner, JoachimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Glaeser, SvenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kahn, NicolasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Welte, TobiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Neurohr, ClausUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schwaiblmair, MartinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bahmer, ThomasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Oqueka, TimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Frankenberger, MarionUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kreuter, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
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:
KeywordsLanguage
CLINICAL-PRACTICE; DOUBLE-BLIND; PIRFENIDONE; ACETYLCYSTEINE; MANAGEMENT; THERAPY; SAFETYMultiple languages
Respiratory SystemMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/32430

Downloads

Downloads per month over past year

Altmetric

Export

Actions (login required)

View Item View Item