肺纤维化的发病机制及其治疗药物研究进展

Pathogenesis of Pulmonary Fibrosis and Progress in Drug Development

肺纤维化(PF)是一种慢性、进行性、纤维化性肺疾病,可导致患者肺功能不可逆下降、进行性呼吸衰竭,甚至死亡。PF的发病机制尚未完全阐明,目前研究表明PF是由于持续的肺泡上皮细胞损伤和修复异常,成纤维细胞的增生,细胞外基质的积聚,导致肺部结构紊乱,形成纤维化。近年来随着对其发病机制的了解,全球已有2个PF治疗药物(吡非尼酮和尼达尼布)被批准上市,十多个PF治疗药物处于临床研究阶段。综述与肺纤维化相关的发病机制及其最新治疗药物临床研究进展。

Pulmonary fibrosis (PF) is a chronic, progressive, fibrotic lung disease that causes irreversible decline in lung function, progressive respiratory failure, and even death. The pathogenesis of PF has not been fully elucidated. Current studies have shown that pulmonary fibrosis is characterized by repetitive microscopic alveolar epithelial cell injury and dysregulated repair, fibroblast proliferation and excessive deposition of extracellular matrix (ECM), resulting in loss of parenchymal architecture and fibrosis. In recent years, with increased understanding of PF pathogenesis, two therapeutic drugs (pirfenidone and nintedanib) have been approved for marketing worldwide, and more than ten are under clinical decelopment. This paper reviewed the pathogenesis associated with pulmonary fibrosis and the progress of clinical researches on the latest therapeutic drugs.