特发性肺纤维化发病机制的研究进展

Research Progress in Pathogenesis Mechanism of Idiopathic Interstitial Pulmonary Fibrosis

特发性肺纤维化(IPF)是最常见的特发性间质性肺炎,随着全球老龄人口的增加,其发病率和患病率呈上升趋势。对IPF的诊疗在一定程度上减缓了疾病进展,但尚不能完全治愈。IPF的发病机制与早期肺泡上皮细胞的反复损伤以及异常修复有关,多种细胞因子、信号通路、表观遗传学等因素亦起着重要作用,但具体病因和发病机制目前尚不明确。IPF诊断较困难,患者预后不良,成为近年来国内外研究的热点和难点。

Idiopathic pulmonary fibrosis( IPF) is the most common idiopathic interstitial pneumonia. With the increase of the global aging population,the incidence and prevalence of IPF are on the rise. The diagnosis and treatment of IPF have slowed down the progress of the disease to a certain extent,but complete cure cannot be achieved. The pathogenesis of IPF is related to the early repeated injury and abnormal repair of alveolar epithelial cells,while a variety of cytokines,signaling pathways,epigenetics and other factors also play important roles,but the specific etiology and pathogenesis are still unclear.IPF is difficult to diagnose and has a poor prognosis,which has become a hotspot and challenge in the research field both in and out of China in recent years.