Long-term overall survival and progression-free survival in idiopathic pulmonary fibrosis treated by pirfenidone or nintedanib or their switch. Real world data from the EMPIRE registry

Abstract

Aim: Treatment either with nintedanib (N) or pirfenidone (P) can slow progression of idiopathic pulmonary fibrosis (IPF). Long term effect of this treatment in the real world has not been adequately documented to date. We aimed to compare the overall survival (OS) and progression free survival (PFS) in the patients from the European MultiPartner IPF registry (EMPIRE) treated either by P or N or switch from either N to P or P to N (S) with other or no treatment (O). Patients and Methods: 2745 IPF patients from the EMPIRE registry (809 F and 1936 M, mean age at diagnosis 66.5 years) were enrolled to the retrospective study (821 on P, 637 on N, 148 S and 1139 O). Progression was defined as death, or transplantation, or decline of FVC ˃ 10% or TLCO ˃ 15% compared to previous value. OS and PFS in the subgroups were counted and compared. Results: Median OS in months was from the entry to the registry: 56.7 P, 84.7 N, 76.9 S, 37.5 O; 5-years survival rate was: 0.461 P, 0.622 N, 0.65 S, 0.333 O and 10-years survival rate was: 0.216 P, 0.364 N, 0.491 S and 0.139 O (p ˂0.001). Median PFS in months was from the entry to the registry: 20.5 P, 22.9 N, 30.4 S and 17.3 O, 5-years PFS rate was: 0.173 P, 0.232 N, 0.333 S, 0.094 O and 10-years PFS rate was: 0.04 P, 0.055 N, 0.084 S and 0.008 O (p ˂0.001). Conclusion: The results show a consistent effect of antifibrotic treatment on OS and PFS in a large cohort of the real-world IPF patients. Moreover, the effect of a switch of antifibrotics on OS and PFS is even higher than either P or N alone. The EMPIRE registry is supported by Boehringer Ingelheim