Approved and Experimental Therapies for Idiopathic Pulmonary Fibrosis

Purpose of Review Treatment of idiopathic pulmonary fibrosis (IPF) shifted significantly in the past few years, in the light of advances in understanding pathological pathways. The promising results coming from recent trials led to a growing number of studies dealing with IPF. The purpose of this article is to review scientific evidences supporting approved therapies and to provide an overview of experimental treatments that are currently under investigation. Recent Findings Approved anti-fibrotic treatments are based on large randomized trials showing the efficacy of pirfenidone and nintedanib in slowing IPF progression with an acceptable tolerability. However, we are still far from a definite cure and research efforts are focused on exploring new therapeutic targets and alternative treatment strategies. Summary Although pirfenidone and nintedanib remain the mainstay of IPF treatment, novel therapies are under investigation in clinical trials. Combination therapy will probably be a successful key for the future, targeting multiple pathways simultaneously. Clinical Registration NCT01619085