特发性肺纤维化相关信号通路及治疗药物研究进展

特发性肺纤维化是一种慢性的进行性肺部疾病，它也是特发性间质性肺炎最常见的形式之一，具有很高死亡率，自诊断之日起中位生存期仅为2～3年。尽管其发病机制尚不明确，但已发现多条信号通路在该疾病发生发展过程中起重要作用。目前FDA批准用于治疗特发性肺纤维化的药物主要有吡非尼酮和尼达尼布，另外还有10余种药物处于临床研究阶段。综述与特发性肺纤维化相关的信号通路及其临床在研药物的研究进展。

Advances in Research on Idiopathic Pulmonary Fibrosis Related Signaling Pathways and Therapeutic Drugs

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease. It is one of the most common forms of idiopathic interstitial pneumonia with high mortality rate. The median survival time from diagnosis is only 2 to 3 years. Though the pathogenesis of IPF is still unclear, it has been found that some signaling pathways play important roles in the occurrence and development of IPF. Currently, pirfenidone and nintedanib are the main drugs approved by FDA for the treatment of IPF, and more than 10 drugs are in clinical studies. This article reviews the research progress of IPF related signaling pathways and the drugs in clinical development.