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

Authors	ZURKOVA Monika KRIEGOVA Eva KOLEK Vitezslav LOSTAKOVA Vladimira STERCLOVA Martina BARTOS Vladimir DOUBKOVÁ Martina BINKOVÁ Ilona SVOBODA Michal STRENKOVÁ Jana JANOTOVA Marketa PLACKOVA Martina LACINA Ladislav RIHAK Vladimir PETRIK Frantisek LISA Pavlina BITTENGLOVA Radka TYL Richard ONDREJKA Gustav SULDOVA Hana LNENICKA Jaroslav PSIKALOVA Jana SNIZEK Tomas HOMOLKA Jiri KRALOVA Renata KERVITZER Jan VASAKOVA Martina
Year of publication	2019
Type	Article in Periodical
Magazine / Source	RESPIRATORY RESEARCH
MU Faculty or unit	Faculty of Medicine
Citation
Web	http://dx.doi.org/10.1186/s12931-019-0977-2
Doi	http://dx.doi.org/10.1186/s12931-019-0977-2
Keywords	Idiopathic pulmonary fibrosis; Pirfenidone; Mortality prediction; Disease progression
Description	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. 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. During a 2-yr follow-up, less than a quarter of the patients progressed on pirfenidone as assessed by the decline of ae10% FVC (17.0%) and ae 15% DLCO (14.3%). On pirfenidone, the DLCO (ae10%) declines at 6, 12, 18 and 24 months' and DLCO (ae15%) 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). 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 ae10% shows a potential as a mortality predictor in IPF patients on pirfenidone, and should be routinely evaluated during follow-up examinations.