Abstract
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.
- Idiopathic pulmonary fibrosis
- meta-analysis
- N-acetylcysteine
- pirfenidone
- progression-free survival
- vital capacity
Footnotes
Following the presentation sponsored by InterMune Inc. at the 2010 ERS Annual Congress (Barcelona, Spain), pirfenidone has received marketing authorisation in the European Union in 2011.
Provenance
Publication of this peer-reviewed article was supported by InterMune Inc., Brisbane, CA, USA (article sponsor, European Respiratory Review issue 121).
Statement of Interest
U. Costabel has received consultancy fees from Actelion, Boehringer Ingelheim, Centocor, Gilead and InterMune, and has received lecture fees from InterMune.
- Received February 15, 2011.
- Accepted March 3, 2011.
- ©ERS 2011