Abstract
Background: Antifibrotic treatment(Nintedanib(N) and Pirfenidon(P)) slows down lung function decline in patients with idiopathic pulmonary fibrosis (IPF). Recently, N was also approved for non-IPF progressive pulmonary fibrosis (PPF). However, real-world data on antifibrotics in PPF are scarce. Thus, we evaluated lung function trajectory before and after start of treatment in patients with IPF and other PPF.
Methods: This multicentre registry study included patients with IPF and PPF. Piecewise linear mixed models were used to analyse differences in FVC and DLCO 12 months before and after start of treatment.
Results: 152 IPF(66%N,33%P) and 33 PPF(84%N,15%P) patients were included. In IPF, treatment tended to improve FVC (before:∆=-0.09, p=0.15, after ∆=0.01, p=0.73), and reduced decline of DLCO(p=0.01). In PPF, both FVC (before: ∆=-0.51, p<0.005, after: ∆0.09, p=0.27) and DLCO (before: ∆-1.17, p=<0.005, after: ∆-0.20, p=0.19) stabilized after treatment. FVC and DLCO were lower in PPF at time of treatment initiation. Hereafter, slopes between both groups did not differ (FVC: p=0.24, DLCO: p=0.37)(F1).
Conclusion: These real-world data confirm that antifibrotic treatment stabilizes lung function in both IPF and PPF. Patients with IPF started treatment at more preserved lung function. Further research is needed to optimize timing of antifibrotics in patients with PPF.
Footnotes
Cite this article as Eur Respir J 2022; 60: Suppl. 66, 4442.
This article was presented at the 2022 ERS International Congress, in session “-”.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2022
