There is a lack of data on the long-term effect of nintedanib on survival in specific
groups of idiopathic pulmonary fibrosis (IPF) patients with different phenotypes.
We investigated the outcomes of nintedanib therapy in an observational study of a
large multicentre real-world cohort of IPF patients with various initial characteristics.The
analysis included IPF patients treated with nintedanib (NIN) and IPF patients not
receiving antifibrotic treatment (NAF) enrolled for the EMPIRE registry in 2015-2020.
The patients were stratified according to their initial FVC predicted, dyspnoea, UIP
pattern and age. All-cause mortality and annual rate of FVC decline were the main
endpoints. Cox proportional hazards model for survival assessment and linear mixed-effects
model for FVC decline modelling were used.A total of 869 NIN patients and 691 NAF
patients were eligible for the analysis. The annual FVC decline rate was significantly
different (adjusted values -0.053 l/yr vs -0.122 l/yr; p = 0.001). The adjusted hazard
ratio (HR) for mortality was 0.40 (95% CI 0.3 to 0.53, p < 0.001). The most significant
effect of nintedanib was demonstrated in patients with impaired lung function, i.e.,
with an FVC predicted to be less than 80% and a NYHA II to IV. Nintedanib therapy
also reduced the difference in survival between men and women.Modelling confirmed
that NIN therapy reduced differences in OS between patients with better and worse
initial conditions and prognosis. Our results indicate that NIN is particularly beneficial
for patients with advanced IPF and more severe phenotypes.EMPIRE was registered as
a non-interventional post-registration study at the State Institute for Drug Control
of the Czech Republic under ID 1412080000 on 8 December 2014.
