Pirfenidone for the treatment of idiopathic pulmonary fibrosis: therapeutic potential prompts further investigation

Idiopathic pulmonary fibrosis (IPF) is a rare, progressive disease of the lungs with unknown aetiology. Currently, there is no therapy that is specifically approved to be used for IPF treatment and the efficacy of 'conventional therapy , recommended by the existing therapeutic guidelines and consisting of a combination of corticosteroids with immunosuppressives, is not sufficiently substantiated. Based on the current pathogenetic hypothesis advocating the major role of fibrosis in IPF, novel antifibrotic agents are being developed for the treatment of this disease. Among them, IFN- and N-acetylcysteine are at later stages of clinical development. Pirfenidone is another antifibrotic agent that has also demonstrated preclinical anti-inflammatory and antioxidant effects. Earlier clinical studies showed that prifenidone could be efficacious for IPF treatment. Pirfenidone acquired orphan drug status in both Europe and the US for the treatment of IPF. The current randomised, placebo-controlled study authored by Azuma et al. further assesses its efficacy in IPF patients and searches for new potential efficacy end points in this setting.