Title: Pirfenidone in patients with non-IPF progressive fibrotic interstitial lung diseases: expert guidance is urgently needed
Abstract: It has been proposed that patients with fibrotic lung diseases other than idiopathic pulmonary fibrosis (IPF) that progress despite conventional management (ie, the progressive fibrotic phenotype) are likely to have pathogenetic mechanisms in common with IPF, potentially responding to antifibrotic therapies. 1 Wells AU Brown KK Flaherty KR Kolb M Thannickal VJ IPF Consensus Working GroupWhat's in a name? That which we call IPF, by any other name would act the same. Eur Respir J. 2018; 511800692 Crossref PubMed Scopus (116) Google Scholar This was the conceptual basis of the successful INBUILD trial 2 Flaherty KR Wells AU Cottin V et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019; 381: 1718-1727 Crossref PubMed Scopus (457) Google Scholar of nintedanib for progressive fibrotic interstitial lung diseases (ILDs) other than IPF, and there has been considerable interest in whether pirfenidone is equally efficacious in these difficult-to-treat diseases. The first pirfenidone trial, in patients with fibrotic non-specific interstitial pneumonia or unclassifiable ILD, was deemed a negative study, on the basis of a non-validated primary endpoint (ie, home spirometric measurements, analysed parametrically and confounded by outlying observations in patients remaining in the study for short periods). 3 Maher TM Corte TJ Fischer A et al. Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial. Lancet Respir Med. 2020; 8: 147-157 Summary Full Text Full Text PDF PubMed Scopus (156) Google Scholar However, many experts view this study as providing convincing evidence of pirfenidone efficacy versus placebo in patients with a progressive fibrotic phenotype; a key secondary endpoint, serial forced vital capacity (FVC) measured in lung function laboratories, which equated to the primary endpoint in INBUILD, was unequivocally positive. Thus, the results of the RELIEF trial of pirfenidone, presented by Jürgen Behr and colleagues in the Lancet Respiratory Medicine, 4 Behr J Prasse A Kreuter M et al. Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial. Lancet Respir Med. 2021; (published online March 29.)https://doi.org/10.1016/S2213-2600(20)30532-4 Summary Full Text Full Text PDF PubMed Scopus (18) Google Scholar in patients with progressive fibrotic ILDs other than IPF, have been eagerly anticipated. Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trialIn view of the premature study termination, results should be interpreted with care. Nevertheless, our data suggest that in patients with fibrotic ILDs other than IPF who deteriorate despite conventional therapy, adding pirfenidone to existing treatment might attenuate disease progression as measured by decline in FVC. Full-Text PDF
Publication Year: 2021
Publication Date: 2021-03-31
Language: en
Type: article
Indexed In: ['crossref']
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Cited By Count: 5
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