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.

Department of Immunology Faculty of Medicine and Dentistry University Hospital Olomouc Palacky University in Olomouc Hnevotinska 3 775 15 Olomouc Czech Republic

Department of Pulmonary Diseases and Tuberculosis Faculty of Medicine and Dentistry Palacky University in Olomouc University Hospital Olomouc Olomouc Czech Republic

Department of Pulmonary Diseases and Tuberculosis Faculty of Medicine at Masaryk University University Hospital Brno Brno Czech Republic

Department of Pulmonary Diseases and Tuberculosis Faculty of Medicine at University of Ostrava University Hospital Ostrava Ostrava Czech Republic

Department of Pulmonary Diseases and Tuberculosis Regional Medical Association JSC Masaryk Hospital in Usti nad Labem Usti nad Labem Czech Republic

Department of Pulmonary Medicine 2nd Faculty of Medicine at Charles University Prague University Hospital in Motol Prague Czech Republic

Department of Pulmonary Medicine and Allergology Hospital Kromeriz Kromeriz Czech Republic

Department of Pulmonary Medicine and Thoracic Surgery Hospital Na Bulovce Prague Czech Republic

Department of Pulmonary Medicine Faculty of Medicine in Hradec Kralove at Charles University Prague University Hospital Hradec Kralove Prague Czech Republic

Department of Pulmonary Medicine Hospital Ceske Budejovice Ceske Budejovice Czech Republic

Department of Pulmonary Medicine Hospital Jihlava Jihlava Czech Republic

Department of Pulmonary Medicine Hospital Novy Jicin Novy Jicin Czech Republic

Department of Pulmonary Medicine Hospital Znojmo Znojmo Czech Republic

Department of Pulmonary Medicine Regional Hospital Pardubice Pardubice Czech Republic

Department of Pulmonary Medicine Tomas Bata Regional Hospital Zlin Czech Republic

Department of Pulmonary Medicine University Hospital Plzen Pilsen Czech Republic

Department of Respiratory Medicine 1st Faculty of Medicine Charles University Prague Thomayer Hospital Prague Czech Republic

Institute of Biostatistics and Analyses Faculty of Medicine Masaryk University Brno Czech Republic

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