Effect of pirfenidone on lung function decline and survival: 5-yr experience from a real-life IPF cohort from the Czech EMPIRE registry.

ABSTRACT: INTRODUCTION:Pirfenidone, an antifibrotic drug, slows-down the disease progression in idiopathic pulmonary fibrosis (IPF) over 12?months, however limited data on the decline of lung function and overall survival (OS) in real-world cohorts on longer follow-up exists. PATIENTS/METHODS:Of the enrolled Czech IPF patients (n?=?841) from an EMPIRE registry, 383 (45.5%) received pirfenidone, 218 (25.9%) no-antifibrotic treatment and 240 (28.5%) were excluded (missing data, nintedanib treatment). The 2- and 5-yrs OS and forced vital capacity (FVC) and diffusing lung capacity for carbon monoxide (DLCO) were investigated at treatment initiation and 6, 12, 18 and 24?months' follow-up. RESULTS:During a 2-yr follow-up, less than a quarter of the patients progressed on pirfenidone as assessed by the decline of ?10% FVC (17.0%) and???15% DLCO (14.3%). On pirfenidone, the DLCO (?10%) declines at 6, 12, 18 and 24?months' and DLCO (?15%) declines at 6, 18 and 24?months' follow-up were associated with increased mortality. The DLCO decline showed higher predictive value for mortality than FVC decline. In patients with no-antifibrotics, FVC and DLCO declines were not predictive for mortality. Pirfenidone increased 5-yrs OS over no-antifibrotic treatment (55.9% vs 31.5% alive, P?=?0.002). CONCLUSION:Our study observed the 2-yrs sustained effect of pirfenidone on the decline of lung function and survival in the real-world patient's IPF cohort. DLCO decline of ?10% shows a potential as a mortality predictor in IPF patients on pirfenidone, and should be routinely evaluated during follow-up examinations.

SUBMITTER: Zurkova M 

PROVIDER: S-EPMC6341650 | biostudies-other | 2019 Jan

REPOSITORIES: biostudies-other