• Antoni Xaubet, Anna Serrano-Mollar, and Julio Ancochea.
• 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.
• Expert Opin Pharmacother. 2014 Feb 1;15(2):275-81.

IntroductionIdiopathic 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.Areas CoveredThis 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.Expert OpinionPirfenidone 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.

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