Publication details

Survival and lung function decline in patients with definite, probable and possible idiopathic pulmonary fibrosis treated with pirfenidone

Authors	MÁJEK Ondřej GREGOR Jakub MOGULKOĆ Nesrin LEWANDOWSKA Katarzyna ŠTERCLOVÁ Martina MÜLLER Veronika HÁJKOVÁ Marta KRAMER Mordechai R TEKAVEC-TRKANJEC Jasna JOVANOVIĆ Dragana STUDNICKA Michael STOEVA Natalia KLAUS-UWE Kirchgässler LITTNEROVÁ Simona DUŠEK Ladislav VAŠÁKOVÁ KOZIAR Martina
Year of publication	2022
Type	Article in Periodical
Magazine / Source	PLOS One
MU Faculty or unit	Faculty of Medicine
Citation
Web	https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0273854
Doi	http://dx.doi.org/10.1371/journal.pone.0273854
Keywords	idiopathic pulmonary fibrosis; pirfenidone; survival ; lung function decline
Description	Background There is no clear evidence whether pirfenidone has a benefit in patients with probable or possible UIP, i.e. when idiopathic pulmonary fibrosis (IPF) is diagnosed with a lower degree of diagnostic certainty. We report on outcomes of treatment with pirfenidone in IPF patients diagnosed with various degrees of certainty. Methods and findings We followed patients in the multi-national European MultiPartner IPF Registry (EMPIRE) first seen between 2015 and 2018. Patients were assessed with HRCT, histopathology and received a multi-disciplinary team (MDT) IPF diagnosis. Endpoints of interest were overall survival (OS), progression-free survival (PFS) and lung function decline. Results A total of 1626 patients were analysed, treated with either pirfenidone (N = 808) or receiving no antifibrotic treatment (N = 818). When patients treated with pirfenidone were compared to patients not receiving antifibrotic treatment, OS (one-, two- and three-year probability of survival 0.871 vs 0.798; 0.728 vs 0.632; 0.579 vs 0.556, P = 0.002), and PFS (one-, two- and three-year probability of survival 0.597 vs 0.536; 0.309 vs 0.281; 0.158 vs 0.148, P = 0.043) was higher, and FVC decline smaller (-0.073 l/yr vs -0.169 l/yr, P = 0.017). The benefit of pirfenidone on OS and PFS was also seen in patients with probable or possible IPF. Conclusions This EMPIRE analysis confirms the favourable outcomes observed for pirfenidone treatment in patients with definitive IPF and indicates benefits also for patients with probable or possible IPF.