Advances in pharmacotherapy for fibrotic interstitial lung disease

Abstract Fibrotic interstitial lung diseases (ILDs), including idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF), are characterized by an irreversible decline in lung function and high mortality. While two approved antifibrotic therapies, nintedanib and pirfenidone, have been shown to alter the disease trajectory by slowing its progression, the disease remains incurable. This review summarizes current pharmacological treatments for fibrotic interstitial lung disease (f-ILD), mainly IPF and PPF; highlights novel antifibrotic targets such as the lysophosphatidic acid (LPA) receptor; outlines the therapeutic development pipeline; and discusses emerging strategies, including personalized medicine and early intervention.