Pirfenidone: antifibrotic agent for idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a devastating disease with rare incidence but high mortality, and the pathogenesis of which is still poorly understood. Available treatment options have been empirically applied but evidence-based benefits have not yet been confirmed. Pirfenidone is an antifibrotic agent that is potentially effective for IPF treatment. Preclinical studies have been reported using experimental animal models, which revealed inhibitory effects pn profibrotic and proinflammatory cytokines. Several clinical studies provided promising and reproducible effects for inhibition of IPF disease progression in different nations. The efficacy is demonstrated in patients with mild-to-moderate impairment of pulmonary functions, but not confirmed for patients with severe impairment. Major adverse events are photosensitivity and anorexia, but the treatment was generally safe and well tolerated. In this article, the usefulness and limitations of pirfenidone in IPF treatment are discussed to determine its potential for the management of IPF progression.