Abstract
Idiopathic 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.
Financial & competing interests disclosure
Roland M du Bois has received consulting fees/honoraria from Boehringer Ingelheim, InterMune, Actelion and Bayer; Roland M du Bois has also obtained speakers fees from InterMune, Actellion and GlaxoSmithKline, and educational presentation payment from InterMune. Luca Richeldi has received consulting fees/honoraria from InterMune, Celgene, Gilead and Boehringer Ingelheim, and speaker’s fees from InterMune. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.
No writing assistance was utilized in the production of this manuscript.