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.