PURPOSE OF REVIEW: Since 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 FINDINGS: This 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. SUMMARY: Despite 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.
PURPOSE OF REVIEW: Since 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 FINDINGS: This 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. SUMMARY: Despite 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.
Authors: Chi Hung; Geoffrey Linn; Yu-Hua Chow; Akio Kobayashi; Kristen Mittelsteadt; William A Altemeier; Sina A Gharib; Lynn M Schnapp; Jeremy S Duffield Journal: Am J Respir Crit Care Med Date: 2013-10-01 Impact factor: 21.405
Authors: Sushravya Raghunath; Srinivasan Rajagopalan; Ronald A Karwoski; Fabien Maldonado; Tobias Peikert; Teng Moua; Jay H Ryu; Brian J Bartholmai; Richard A Robb Journal: PLoS One Date: 2014-03-27 Impact factor: 3.240
Authors: Anna Stainer; Paola Faverio; Sara Busnelli; Martina Catalano; Matteo Della Zoppa; Almerico Marruchella; Alberto Pesci; Fabrizio Luppi Journal: Int J Mol Sci Date: 2021-06-10 Impact factor: 5.923