Pharmacological treatment of idiopathic pulmonary fibrosis and fibrosing interstitial lung diseases: current trends and future directions

Idiopathic pulmonary fibrosis (IPF) was considered untreatable until the development of therapeutic drugs and diagnostic technology that made it possible to slow the progression of IPF. In 2014, pirfenidone and nintedanib were approved simultaneously as therapeutic drugs for patients with IPF. These drugs have proven effective in reducing further progression of pulmonary fibrosis, acute exacerbation, and mortality and have consistent effects regardless of the severity of IPF. The indications of nintedanib and pirfenidone are gradually expanding for various other diseases that cause pulmonary fibrosis. Currently, IPF pathogenesis is associated with the type 2 alveolar epithelium and repeated or persistent damage to its cells. Such damage may induce an abnormal wound healing response, causing fibrosis rather than repair. Several promising drugs have been developed for reducing or reversing fibrosis, each with a different molecular target implicated in pulmonary fibrosis. Due to the heterogeneous mechanisms underlying pulmonary fibrosis, future disease management is likely to comprise combinations of therapies targeting a range of disease mechanisms.