• Fabrizio Luppi, Paolo Spagnolo, Stefania Cerri, and Luca Richeldi.
• Center for Rare Lung Diseases, University Hospital of Modena, Modena, Italy.
• Curr Opin Pulm Med. 2012 Sep 1; 18 (5): 428-32.

Purpose Of ReviewSince the late 1990 s, when a more uniform definition of idiopathic pulmonary fibrosis (IPF) was proposed, more than 3000 patients have been enrolled in clinical studies exploring novel therapies. Some of the most relevant trials have been published only recently.Recent FindingsThis review describes and comments on the randomized clinical trials in IPF published within the past 18 months, with emphasis on the studies evaluating pirfenidone, which at present is the only drug approved for IPF (at least in Europe and Japan), and bosentan. This latter represents the largest single trial performed in IPF so far.SummaryDespite multiple clinical trials, there is no strong, definitive evidence in favor of any agent to treat IPF. On the other hand, the placebo arms of these large trials have provided us with important critical information on the natural history of this disease. Clinical heterogeneity represents a critical issue to be taken into account in designing future clinical trials. The limited effectiveness of current treatment regimes has fuelled the search for a variety of new therapeutic approaches.

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