Emerging potential treatments: new hope for idiopathic pulmonary fibrosis patients?

Currently, there are no approved pharmacological treatments for the management of patients with idiopathic pulmonary fibrosis (IPF) in the USA or Europe. Pirfenidone is an orally bio-available small molecule that exhibits antifibrotic and anti-inflammatory properties in a variety of in vitro and ani...

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Main Author: U. Costabel
Format: Article
Language:English
Published: European Respiratory Society 2011-09-01
Series:European Respiratory Review
Subjects:
Online Access:http://err.ersjournals.com/content/20/121/201.full.pdf+html
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author U. Costabel
author_facet U. Costabel
author_sort U. Costabel
collection DOAJ
description Currently, there are no approved pharmacological treatments for the management of patients with idiopathic pulmonary fibrosis (IPF) in the USA or Europe. Pirfenidone is an orally bio-available small molecule that exhibits antifibrotic and anti-inflammatory properties in a variety of in vitro and animal models. Pirfenidone has been evaluated in four randomised, double-blind, placebo-controlled clinical trials conducted in Japan, North America and Europe. The totality of the data from these trials indicates that pirfenidone is able to reduce the rate of decline in lung function, measured as change in per cent predicted forced vital capacity (FVC) or vital capacity. There was also an effect on secondary end-points of progression free survival, categorical change in per cent predicted FVC, and the 6-min walk test. A recent meta-analysis of the three phase III studies in IPF demonstrated that pirfenidone significantly reduced the risk of disease progression by 30%. The efficacy of pirfenidone is associated with an acceptable tolerability and safety profile.
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spelling doaj.art-135646e6108a46aba6b2faff8f1072952022-12-22T03:29:05ZengEuropean Respiratory SocietyEuropean Respiratory Review0905-91801600-06172011-09-0120121201207Emerging potential treatments: new hope for idiopathic pulmonary fibrosis patients?U. CostabelCurrently, there are no approved pharmacological treatments for the management of patients with idiopathic pulmonary fibrosis (IPF) in the USA or Europe. Pirfenidone is an orally bio-available small molecule that exhibits antifibrotic and anti-inflammatory properties in a variety of in vitro and animal models. Pirfenidone has been evaluated in four randomised, double-blind, placebo-controlled clinical trials conducted in Japan, North America and Europe. The totality of the data from these trials indicates that pirfenidone is able to reduce the rate of decline in lung function, measured as change in per cent predicted forced vital capacity (FVC) or vital capacity. There was also an effect on secondary end-points of progression free survival, categorical change in per cent predicted FVC, and the 6-min walk test. A recent meta-analysis of the three phase III studies in IPF demonstrated that pirfenidone significantly reduced the risk of disease progression by 30%. The efficacy of pirfenidone is associated with an acceptable tolerability and safety profile.http://err.ersjournals.com/content/20/121/201.full.pdf+htmlIdiopathic pulmonary fibrosismeta-analysisN-acetylcysteinepirfenidoneprogression-free survivalvital capacity
spellingShingle U. Costabel
Emerging potential treatments: new hope for idiopathic pulmonary fibrosis patients?
European Respiratory Review
Idiopathic pulmonary fibrosis
meta-analysis
N-acetylcysteine
pirfenidone
progression-free survival
vital capacity
title Emerging potential treatments: new hope for idiopathic pulmonary fibrosis patients?
title_full Emerging potential treatments: new hope for idiopathic pulmonary fibrosis patients?
title_fullStr Emerging potential treatments: new hope for idiopathic pulmonary fibrosis patients?
title_full_unstemmed Emerging potential treatments: new hope for idiopathic pulmonary fibrosis patients?
title_short Emerging potential treatments: new hope for idiopathic pulmonary fibrosis patients?
title_sort emerging potential treatments new hope for idiopathic pulmonary fibrosis patients
topic Idiopathic pulmonary fibrosis
meta-analysis
N-acetylcysteine
pirfenidone
progression-free survival
vital capacity
url http://err.ersjournals.com/content/20/121/201.full.pdf+html
work_keys_str_mv AT ucostabel emergingpotentialtreatmentsnewhopeforidiopathicpulmonaryfibrosispatients