Pirfenidone for idiopathic pulmonary fibrosis: analysis of pooled data from three multinational phase 3 trials
Paul W. Noble,Carlo Albera,Williamson Z. Bradford,Ulrich Costabel,Roland M. du Bois,Elizabeth A. Fagan,Robert S. Fishman,Ian Glaspole,Marilyn K. Glassberg,Lisa Lancaster,David J. Lederer,Jonathan A. Leff,Steven D. Nathan,Carlos Alberto de Castro Pereira,Jeffrey J. Swigris,Dominique Valeyre,Talmadge E. King +16 more
TLDR
Analysis of data from three phase 3 trials demonstrated that treatment with pirfenidone for 1 year resulted in clinically meaningful reductions in disease progression in patients with IPF.Abstract:
Pirfenidone is an antifibrotic agent that has been evaluated in three multinational phase 3 trials in patients with idiopathic pulmonary fibrosis (IPF). We analysed pooled data from the multinational trials to obtain the most precise estimates of the magnitude of treatment effect on measures of disease progression.All patients randomised to pirfenidone 2403 mg·day(-1) or placebo in the CAPACITY or ASCEND studies were included in the analysis. Pooled analyses of outcomes at 1 year were based on the pre-specified end-points and analytic methods described in the ASCEND study protocol.A total of 1247 patients were included in the analysis. At 1 year, pirfenidone reduced the proportion of patients with a ≥10% decline in per cent predicted forced vital capacity or death by 43.8% (95% CI 29.3-55.4%) and increased the proportion of patients with no decline by 59.3% (95% CI 29.0-96.8%). A treatment benefit was also observed for progression-free survival, 6-min walk distance and dyspnoea. Gastrointestinal and skin-related adverse events were more common in the pirfenidone group, but rarely led to discontinuation.Analysis of data from three phase 3 trials demonstrated that treatment with pirfenidone for 1 year resulted in clinically meaningful reductions in disease progression in patients with IPF.read more
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Journal ArticleDOI
Idiopathic pulmonary fibrosis
TL;DR: This disease has improved understanding of the mechanisms of lung fibrosis, and offers hope that similar approaches will transform the management of patients with other progressive fibrotic lung diseases.
Journal ArticleDOI
Idiopathic pulmonary fibrosis
Fernando J. Martinez,Harold R. Collard,Annie Pardo,Ganesh Raghu,Luca Richeldi,Moisés Selman,Jeffrey J. Swigris,Hiroyuki Taniguchi,Athol U. Wells +8 more
TL;DR: The biological processes underlying IPF are thought to reflect an aberrant reparative response to repetitive alveolar epithelial injury in a genetically susceptible ageing individual, although many questions remain on how to define susceptibility.
Journal ArticleDOI
Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial.
Toby M. Maher,Toby M. Maher,Tamera J. Corte,Tamera J. Corte,Aryeh Fischer,Aryeh Fischer,Michael Kreuter,David J. Lederer,David J. Lederer,Maria Molina-Molina,Judit Axmann,Klaus-Uwe Kirchgaessler,Katerina Samara,Frank Gilberg,Vincent Cottin +14 more
TL;DR: A multicentre, double-blind, randomised, placebo-controlled phase 2 trial to assess the efficacy and safety of pirfenidone in patients with progressive fibrosing unclassifiable ILD, which is characterised by progressive fibrosis of the lung.
Journal ArticleDOI
Effect of pirfenidone on mortality: pooled analyses and meta-analyses of clinical trials in idiopathic pulmonary fibrosis
Steven D. Nathan,Carlo Albera,Williamson Z. Bradford,Ulrich Costabel,Ian Glaspole,Marilyn K. Glassberg,David Kardatzke,Monica Daigl,Klaus Uwe Kirchgaessler,Lisa Lancaster,David J. Lederer,Carlos Alberto de Castro Pereira,Jeffrey J. Swigris,Dominique Valeyre,Paul W. Noble +14 more
TL;DR: Several analytic approaches demonstrated that pirfenidone therapy is associated with a reduction in the relative risk of mortality compared with placebo over 120 weeks.
Journal ArticleDOI
An Open-label, Phase II Study of the Safety and Tolerability of Pirfenidone in Patients with Scleroderma-associated Interstitial Lung Disease: the LOTUSS Trial
Dinesh Khanna,Carlo Albera,Aryeh Fischer,Nader Khalidi,Ganesh Raghu,Lorinda Chung,Dan Chen,Elena Schiopu,Margit Tagliaferri,James R. Seibold,Eduard Gorina +10 more
TL;DR: Pfenidone showed an acceptable tolerability profile in SSc-ILD, although a longer titration may be associated with better tolerability, and exploratory disease outcomes remained largely unchanged.
References
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Journal ArticleDOI
An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-Based Guidelines for Diagnosis and Management
Ganesh Raghu,Harold R. Collard,Jim J. Egan,Fernando J. Martinez,Juergen Behr,Kevin K. Brown,Thomas V. Colby,Jean-François Cordier,Kevin R. Flaherty,Joseph A. Lasky,David A. Lynch,Jay H. Ryu,Jeffrey J. Swigris,Athol U. Wells,Julio Ancochea,Demosthenes Bouros,Carlos Roberto Ribeiro de Carvalho,Ulrich Costabel,Masahito Ebina,David M. Hansell,Takeshi Johkoh,Dong Soon Kim,Talmadge E. King,Yasuhiro Kondoh,Jeffrey L. Myers,Nestor L. Müller,Andrew G. Nicholson,Luca Richeldi,Moisés Selman,Rosalind F. Dudden,Barbara S. Griss,Shandra Protzko,Holger J. Schünemann +32 more
TL;DR: This document represents the current state of knowledge regarding idiopathic pulmonary fibrosis, and contains sections on definition and epidemiology, risk factors, diagnosis, natural history, staging and prognosis, treatment, and monitoring disease course.
Journal ArticleDOI
A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis
Talmadge E. King,Williamson Z. Bradford,Socorro Castro-Bernardini,Elizabeth A. Fagan,Ian Glaspole,Marilyn K. Glassberg,Eduard Gorina,Peter Hopkins,David Kardatzke,Lisa Lancaster,David J. Lederer,Steven D. Nathan,Carlos Alberto de Castro Pereira,Steven A. Sahn,Robert Sussman,Jeffrey J. Swigris,Paul W. Noble +16 more
TL;DR: Pirfenidone, as compared with placebo, reduced disease progression, as reflected by lung function, exercise tolerance, and progression-free survival, in patients with idiopathic pulmonary fibrosis.
Idiopathic Pulmonary Fibrosis
TL;DR: The most common signs and symptoms of idiopathic pulmonary fibrosis are shortness of breath and a persistent dry, hacking cough; many affected individuals also experience a loss of appetite and gradual weight loss.
Journal ArticleDOI
Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials.
Paul W. Noble,Carlo Albera,Williamson Z. Bradford,Ulrich Costabel,Marilyn K. Glassberg,David Kardatzke,Talmadge E. King,Lisa Lancaster,Steven A. Sahn,Javier Szwarcberg,Dominique Valeyre,Roland M. du Bois +11 more
TL;DR: In this article, the CAPACITY program was designed to confirm the results of a phase 2 study that suggested that pirfenidone, a novel antifibrotic and anti-inflammatory drug, reduces deterioration in lung function in patients with idiopathic pulmonary fibrosis.
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