Recent Evidence for Pharmacological Treatment of Idiopathic Pulmonary Fibrosis

Objective: To describe emerging evidence for the pharmacological treatment of idiopathic pulmonary fibrosis (IPF). Data Sources: A search of PubMed (1966 to July 2014) was performed using the terms idiopathic pulmonary fibrosis and treatment. Study Selection and Data Extraction: Review of articles was restricted to articles in English and relating to placebo-controlled or comparative clinical trial data of recent significance. Evidence statements from the most recent international guidelines and some historical trial data were also included for context. Data Synthesis: Numerous treatment options have been evaluated for IPF. Therapies evaluated in large trials have either resulted in increased mortality (anticoagulation, triple-therapy with N-acetylcysteine [NAC], azathioprine, and prednisone) or demonstrated a lack of efficacy (endothelin receptor antagonists, single-agent NAC). Pirfenidone, a novel antifibrotic and anti-inflammatory agent, has demonstrated efficacy in several recent analyses and is the o...