• Monika Zurkova, Eva Kriegova, Vitezslav Kolek, Vladimira Lostakova, Martina Sterclova, Vladimir Bartos, Martina Doubkova, Ilona Binkova, Michal Svoboda, Jana Strenkova, Marketa Janotova, Martina Plackova, Ladislav Lacina, Vladimir Rihak, Frantisek Petrik, Pavlina Lisa, Radka Bittenglova, Richard Tyl, Gustav Ondrejka, Hana Suldova, Jaroslav Lnenicka, Jana Psikalova, Tomas Snizek, Jiri Homolka, Renata Kralova, Jan Kervitzer, Martina Vasakova, ILD section, and IPF registry.
• Department of Pulmonary Diseases and Tuberculosis, Faculty of Medicine and Dentistry, Palacky University in Olomouc, University Hospital Olomouc, Olomouc, Czech Republic.
• Resp Res. 2019 Jan 21; 20 (1): 16.

IntroductionPirfenidone, 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/MethodsOf 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.ResultsDuring 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).ConclusionOur 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.

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