Abstract
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.
Highlights
Pirfenidone, an antifibrotic drug, slows-down the disease progression in idiopathic pulmonary fibrosis (IPF) over 12 months, 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
Lung functions and its decline during treatment in IPF patients Of the patients enrolled into the EMPIRE registry, the data on disease progression and other clinical data was available in 601 patients
The association between forced vital capacity (FVC) and diffusing lung capacity for carbon monoxide (DLCO) declines and mortality in IPF patients we investigated the decline of FVC and DLCO in groups of patients treated with pirfenidone as well as those on no-antifibrotic treatment and its association with mortality at 6, 12, 18 and 24 months
Summary
Pirfenidone, an antifibrotic drug, slows-down the disease progression in idiopathic pulmonary fibrosis (IPF) over 12 months, 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). Idiopathic pulmonary fibrosis (IPF) belongs to the idiopathic interstitial pneumonias; it is characterised by a progressively declining lung function, leading to respiratory failure and death, with a median survival of 2–3 years following initial diagnosis [1, 2]. There is a growing body of evidence that early diagnosis and early initiation of treatment by pirfenidone may preserve lung functional status and prolong life. A positive effect of pirfenidone on disease progression was recently observed in patients with more advanced disease with less preserved lung function [9,10,11,12]
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