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

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) f...

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Published in:	Respiratory research Vol. 20; no. 1; p. 16
Main Authors:	Zurkova, Monika, Kriegova, Eva, Kolek, Vitezslav, Lostakova, Vladimira, Sterclova, Martina, Bartos, Vladimir, Doubkova, Martina, Binkova, Ilona, Svoboda, Michal, Strenkova, 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
Format:	Journal Article
Language:	English
Published:	England BioMed Central 21-01-2019 BMC
Subjects:	Aged Anti-Inflammatory Agents, Non-Steroidal - pharmacology Anti-Inflammatory Agents, Non-Steroidal - therapeutic use Carbon monoxide Clinical trials Cohort Studies Czech Republic - epidemiology Disease Disease control Disease Progression Female Fibrosis Follow-Up Studies Humans Idiopathic pulmonary fibrosis Idiopathic Pulmonary Fibrosis - diagnosis Idiopathic Pulmonary Fibrosis - drug therapy Idiopathic Pulmonary Fibrosis - epidemiology Lung diseases Male Medical prognosis Middle Aged Missing data Mortality Mortality prediction Patients Pirfenidone Pulmonary fibrosis Pyridones - pharmacology Pyridones - therapeutic use Registries Respiratory function Respiratory Function Tests - trends Studies Survival Survival Rate - trends Treatment Outcome Vital Capacity - drug effects Vital Capacity - physiology Czech Republic United Kingdom > UK Eastern Europe Pirfenidone Idiopathic pulmonary fibrosis Mortality prediction Disease progression
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Summary:	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 (DL ) 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 ≥10% FVC (17.0%) and ≥ 15% DL (14.3%). On pirfenidone, the DL (≥10%) declines at 6, 12, 18 and 24 months' and DL (≥15%) declines at 6, 18 and 24 months' follow-up were associated with increased mortality. The DL decline showed higher predictive value for mortality than FVC decline. In patients with no-antifibrotics, FVC and DL 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. DL decline of ≥10% shows a potential as a mortality predictor in IPF patients on pirfenidone, and should be routinely evaluated during follow-up examinations.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	1465-993X 1465-9921 1465-993X 1465-9921
DOI:	10.1186/s12931-019-0977-2