Current advances in idiopathic pulmonary fibrosis: the pathogenesis, therapeutic strategies and candidate molecules

Idiopathic pulmonary fibrosis (IPF) is a type of chronic, progressive lung disease with unknown cause, which is characterized by increasing dyspnea and destruction of lung function with a high mortality rate. Evolving evidence demonstrated that the pathogenesis of IPF involved multiple signaling pathways such as inflammation, oxidative stress and fibrosis. However, drug discovery to prevent or revert IPF has been insufficient to cope with the development. Drug discovery targeting multiple links should be considered. In this review, we will brief the pathogenesis of IPF and discuss several small chemical entities toward the pathogenesis for IPF studied in animal models and clinical trials. The field of novel anti-IPF agents and the future directions for the prevention and treatment of IPF are detailed thoroughly discussed.