Idiopathic pulmonary fibrosis (IPF) is a diffuse parenchymal lung disease with no cure. Up until recently, no treatment had been proven to alter its natural history as judged by rate of lung function decline. In 2014 however, the emergence of two novel anti-fibrotic agents, Pirfenidone and Nintedanib revolutionized the management of this condition. Both have demonstrated the ability to deliver a major reduction in the rate of chronic IPF progression. Areas Covered: This review article focuses on Pirfenidone - a pyridone derivative initially designed as an analgesic and anti-pyretic agent. Here we describe the history of the drug from its inception through to exploratory pre-clinical in-vitro and in-vivo studies where its anti-fibrotic potential was identified, and eventually to large multicenter randomized controlled trials. Expert Commentary: This article also summarizes some of the difficulties surrounding clinical end-point selection in IPF trials and addresses some of the challenges facing the IPF community over the coming years.
Keywords: ASCEND study; CAPACITY study; ILD; IPF; Interstitial lung disease; clinical end points; idiopathic pulmonary fibrosis; lung function; nintedanib; pirfenidone.