• Luca Richeldi and Roland M du Bois.
• University of Modena & Reggio Emilia, Modena, Italy.
• Expert Rev Respir Med. 2011 Aug 1;5(4):473-81.

AbstractIdiopathic pulmonary fibrosis is the most lethal form of diffuse lung fibrosis, killing approximately half of those affected within 2-3 years of diagnosis. Until recently, no therapies had been shown to have an impact on disease progression. The Clinical Studies Assessing Pirfenidone (Esbriet(®)) in IPF: Research of Efficacy and Safety Outcomes (CAPACITY) program comprised two almost identical double-blind placebo-controlled studies assessing the effects of pirfenidone on change in forced vital capacity, the primary end point, over a 72-week period. One of these studies was positive, matching in magnitude the benefit seen in two previous positive Japanese studies. The other study did not meet its primary end point but positive trends were consistent in this and a number of secondary end point indices. Safety was acceptable, comprising mainly problems of tolerability rather than toxicity. It is likely that pirfenidone will be utilised in many countries as first-line therapy and will also be included in studies of combination therapy for this attritional disease.

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