Improved survival of IPF patients treated with antifibrotic drugs compared with untreated patients

Background: Pirfenidone and nintedanib unequivocally slow down FVC decline, but have been inconsistently linked to reduced mortality in phase III studies. Furthermore, there is limited real-world data supporting a potential survival benefit of antifibrotic drugs. Here, transplant-free (TPF) survival of IPF patients receiving antifibrotic drugs (IPF^AF) were compared with an untreated cohort (IPF^non-AF). Methods: This is a single-center observational cohort study using prospectively included patients diagnosed with IPF between 2008-2018. Primary outcomes were TPF survival difference and 1-, 2-, and 3-year cumulative mortality for IPF^AF and IPF^non-AF. This was repeated after GAP stage stratification. Findings: In total, 457 patients were included. The median transplant-free survival was 3.4 years in IPF^AF (n = 313) and 2.2 years in IPF^non-AF (n = 144, p = 0.01). For GAP stage II, a median survival of 3.1 and 1.7 years was noted for IPF^AF (n = 143) and IPF^non-AF (n = 59, p < 0.01), respectively. For GAP stage II and III, decreased 1- and 2-year cumulative mortality was reported for the IPF^AF compared with IPF^non-AF. Noteworthy, more lung cancer related deaths were found in the IPF^non-AF group (8 versus 0, p < 0.01). Interpretation: This large real-world study showed a survival benefit in IPF^AF compared with IPF^non-AF. This especially holds true for patients with GAP stage II and III. Funding: ZonMw TopZorg grant & TZO grant