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) compared with an untreated cohort (IPF)? 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 and IPF. 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 (n = 313) and 2.2 years in IPF (n = 144, p = 0.005). For GAP stage II, a median survival of 3.1 and 1.7 years was noted for IPF (n = 143) and IPF (n = 59, p < 0.001), respectively. A significantly lower 1-, 2-, and 3- year cumulative mortality was found for IPF 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 with GAP III was also significantly lower (19.0% vs 65.0%).

Conclusion: This large real-world study showed a survival benefit in IPF compared with IPF. This especially holds true for patients with GAP stage II and III.