title: Pirfenidone for the treatment of idiopathic pulmonary fibrosis.
abstract: Pirfenidone is an orally administered drug with anti-fibrotic, anti-inflammatory and anti-oxidant properties. The efficacy of pirfenidone is supported by a number of Phase III trials as well as a Cochrane meta-analysis and tolerability data are also provided by clinical trials and a long-term extension phase of these studies. These trials led to the approval of pirfenidone for the treatment of idiopathic pulmonary fibrosis (IPF) in Japan in 2008 and in Europe in 2011 and it is now indicated for treatment of patients with mild-to-moderate IPF. The primary endpoint of these studies has usually been the change in percentage predicted forced vital capacity from baseline; there has been no improvement in respiratory symptoms and/or quality of life measurements and/or decrease in mortality. Clinical and basic research studies are needed to expand our knowledge, understanding the final role of pirfenidone in the treatment of IPF and also identifing genetic factors that influence the effectiveness of this treatment.
authors.affiliation: Pulmonology Unit, Department of Diseases of the Thorax, GB Pierantoni - Morgagni Hospital, via C. Forlanini 34, Forlì, Italy.
authors.forename: Venerino
authors.initials: V
authors.lastname: Poletti
authors.forename: Claudia
authors.initials: C
authors.lastname: Ravaglia
authors.forename: Sara
authors.initials: S
authors.lastname: Tomassetti
mesh_terms: D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000494:pharmacology / Q000627:therapeutic use*; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy*; D011728:Pyridones / Q000494:pharmacology / Q000627:therapeutic use*; D011788:Quality of Life; D012129:Respiratory Function Tests; D016896:Treatment Outcome
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D011728:Pyridones; C093844:pirfenidone
keywords: IPF; idiopathic pulmonary fibrosis; pirfenidone; pulmonary fibrosis; tolerability