title: Pirfenidone for the treatment of idiopathic pulmonary fibrosis.
abstract: BACKGROUND
  Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and lethal fibrosing interstitial pneumonia. The median survival from the onset of the symptoms is 2.8 - 4.2 years and the 5-year survival rate is 20%. Its poor prognosis, combined with the scarcity of treatment options, provides a strong rationale for the development of novel therapeutic strategies. During the past decade there has been a huge rise in clinical trials with anti-fibrotic drugs, although only pirfenidone (Esbriet) has shown a beneficial effect.
  
  
  METHODS
  This article reviews the medical literature on the effectiveness and safety of pirfenidone in IPF, by means of a PubMed search from 1995 to present, completed with some data on file from the manufacturer.
  
  
  CONCLUSIONS
  Pirfenidone is the only anti-fibrotic drug approved for the treatment of IPF. Pirfenidone provides a meaningful clinical effect on reductions in the decrease in forced vital capacity (FVC), six-minute walk test (6MWT) distance and mortality, and it improves the progression-free survival in IPF patients with mild-to-moderate disease. Pirfenidone is well tolerated, with the most common side-effects being gastrointestinal discomfort and photosensitivity. Pirfenidone has a favorable benefit-risk profile and represents a suitable treatment option for patients with mild-to-moderate IPF.
authors.affiliation: Servei de Pneumologia, Hospital Clinic, Universitat de Barcelona Centro de Investigaciones Médicas en Red de Enfermedades Respiratorias (CIBERES) , Villarroel 170, Barcelona 08036 , Spain +60044756 ; +34932275455 ; axaubet@clinic.ub.es.
authors.forename: Antoni
authors.initials: A
authors.lastname: Xaubet
authors.forename: Anna
authors.initials: A
authors.lastname: Serrano-Mollar
authors.forename: Julio
authors.initials: J
authors.lastname: Ancochea
mesh_terms: D000818:Animals; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000009:adverse effects / Q000494:pharmacology / Q000627:therapeutic use*; D018572:Disease-Free Survival; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy* / Q000503:physiopathology; D011379:Prognosis; D011728:Pyridones / Q000009:adverse effects / Q000494:pharmacology / Q000627:therapeutic use*; D018570:Risk Assessment; D012720:Severity of Illness Index; D015996:Survival Rate; D014797:Vital Capacity
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D011728:Pyridones; C093844:pirfenidone