Improved Survival of IPF patients Treated With Antifibrotic Drugs Compared With Untreated Patients

Purpose Pirfenidone and nintedanib unequivocally inhibit FVC decline, but have been inconsistently linked to reduced mortality in phase III studies. On the contrary, real-world data show a survival benefit of antifibrotic drugs. However, it is unknown what this benefit is across different Gender, Age, and Physiology (GAP) stages. Research Questions Is there a difference in transplant-free (TPF) survival of IPF patients receiving antifibrotic drugs (IPF AF ) compared with an untreated cohort (IPF non−AF )? Is this different for patients with GAP stage I, II, or III. 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 stratification for GAP stage. Results 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 = 0.005). 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 < 0.001), respectively. A significantly lower 1-, 2-, and 3- year cumulative mortality was found for IPF AF with GAP stage II (1 yr: 7.0% vs 35.6%, 2 yr: 26.6% vs 55.9%, and 3 yr: 46.9% vs 69.5%). The 1-year cumulative mortality of IPF AF with GAP III was also significantly lower (19.0% vs 65.0%). Conclusion 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.