Advances in the treatment of idiopathic pulmonary fibrosis

Abstract

Introduction: Idiopathic pulmonary fibrosis (IPF) is a lung limited, progressive fibrotic disease with a poor prognosis. The cause is unknown, and currently there is no treatment that reverses the disease or stops progression. This combination of a poor prognosis and the absence of curative therapy has prompted a sustained investigative effort to identify beneficial treatments. Recently released trial results suggest progress.

Areas covered: Although the mechanism of disease is poorly understood, a number of compounds that influence pathways thought to play a mechanistic role have been studied for use in IPF. This article discusses a number of these landmark trials.

Expert opinion: From these studies we conclude that the future treatment of IPF will include expanding pharmacological options. Recent studies have identified two agents that appear to slow disease progression and may offer a window into pathogenesis and future drug targets.

Keywords: Idiopathic pulmonary fibrosis; cryptogenic fibrosing alveolitis; idiopathic interstitial pneumonias; interstitial lung disease; management; therapy; usual interstitial pneumonia.