特發(fā)性肺纖維化(IPF)是一種不可逆、進展性、致死性的慢性肺纖維化疾病,其進展較快,存活率較低,缺乏有效診療手段和治療藥物.近年來隨著對其發(fā)病機制的了解,IPF 治療藥物的開發(fā)也取得了一定進展.全球新批準并上市的治療藥物有吡非尼酮和尼達尼布,處于研發(fā)I、II 期的有10 多種,另外也有一些曾經有希望但已經不推薦用于IPF 治療的藥物.

Idiopathic pulmonary fibrosis (IPF) is a chronic pulmonary fibrosis disease with irreversible, progressive, and fatal phenomenon, characterized by rapid progression and high mortality. And there are lack of effective diagnostic methods and treatment drugs. Recently, with the understanding of its pathogenesis, IPF drug development have also made some progress. Pirfenidone and nintedanib are new drugs approved and listed, and there are more than ten IPF drugs under development. In addition, there are some hope for IPF treatment drugs, but now are not recommended.