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

• Main Author: U. Costabel
• Format: Article
• Language: English
• Published: European Respiratory Society 2011-09-01
• Series: European Respiratory Review
• Subjects: Idiopathic pulmonary fibrosis; meta-analysis; N-acetylcysteine; pirfenidone; progression-free survival; vital capacity
• Online Access: http://err.ersjournals.com/content/20/121/201.full.pdf+html
• ISSN: 0905-9180; 1600-0617

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.