Idiopathic Pulmonary Fibrosis: What Is the Best Treatment?

Abstract

Idiopathic pulmonary fibrosis (IPF) is a disease of chronic progressive interstitial pneumonia limited to the lungs. There is growing interest in this disorder as its incidence has increased over time in most countries around the world. This is likely related to an aging population, increased awareness of the disease, and increasingly sensitive imaging technology. Considerable energy has been devoted to creating an improved understanding of its pathogenesis and developing novel therapies. Although dozens of drugs have been studied for the treatment of IPF, only two, pirfenidone and nintedanib, are currently recommended by international guidelines to slow the disease progression. We review the drugs that have been evaluated as IPF therapy over the past three decades, including the currently recommended pirfenidone and nintedanib, note ongoing clinical trials and provide insights into future directions. (BRN Rev. 2017;3:86-101)