Pirfenidone: antifibrotic agent for idiopathic pulmonary fibrosis

Expert Rev Respir Med. 2010 Jun;4(3):301-10. doi: 10.1586/ers.10.32.


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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Clinical Trials as Topic
  • Disease Progression
  • Drug Approval
  • Humans
  • Idiopathic Pulmonary Fibrosis / drug therapy*
  • Idiopathic Pulmonary Fibrosis / immunology
  • Idiopathic Pulmonary Fibrosis / pathology
  • Idiopathic Pulmonary Fibrosis / physiopathology
  • Lung / drug effects*
  • Lung / immunology
  • Lung / pathology
  • Lung / physiopathology
  • Pyridones / adverse effects
  • Pyridones / pharmacokinetics
  • Pyridones / therapeutic use*
  • Recovery of Function
  • Respiratory System Agents / adverse effects
  • Respiratory System Agents / pharmacokinetics
  • Respiratory System Agents / therapeutic use*
  • Severity of Illness Index
  • Time Factors
  • Treatment Outcome


  • Pyridones
  • Respiratory System Agents
  • pirfenidone