Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive fibrotic lung disease with unclear etiology and pathogenesis.Currently, there is no effective treatment.In the past few decades, multiple clinical trials have been conducted to determine the safety and efficacy of pharmacological treatments for IPF patients.So far, only two drugs, pirfenidone and nintedanib, have been proven to effectively slow down the functional decline and disease progression of IPF, which were approved by the U. S.Food and Drug Administration (FDA) in 2014.In addition, the specific therapeutic effects and mechanisms of a series of anti-fibrotic drugs targeting the pathogenesis of IPF, including connective tissue growth factor, autotaxin, lysophosphatidic acids, and integrin αvβ6, require further investigation.This review summarizes the advances of pharmacological therapy for IPF and provides ideas and basis for the research and development of novel targeted drugs.

Idiopathic pulmonary fibrosis; Drug therapy; Clinical trial