特发性肺纤维化是由不明原因引起的疾病，目前还没有一种药物来有效治疗该疾病。吡非尼酮是一类吡啶类的小分子物质，通过抑制转化生长因子-β，改变胶原合成和累积，抑制细胞外基质增殖和表达，具有抗炎、抗氧化和抗纤维化的作用。在一系列的临床试验中，显示了吡非尼酮是潜在治疗特发性纤维化的药物，具有良好的应用前景。

Idopathic pulmonary fibrosis (IPF) is a disease caused by unknown reason. To date, there is no one drug to effectively treat the disease. Pirfenidone is an anti-inflammatory, anti-oxidant, and antifibrotic angent that inhibits transforming growth factor-β in vitro and changes collagen synthesis and accumulation, and inhibits proliferation and extracellular matrix expression. In a series of clinical trials, pirfenidone is a potential for the treatment of IPF.