原发性胆汁性胆管炎中胆管上皮细胞损伤的机制研究进展

Progress in research of mechanism of biliary epithelial cell injury in primary biliary cholangitis

原发性胆汁性胆管炎(primary biliary cholangitis, PBC)是以慢性胆汁淤积,进行性肝内中、小胆管非化脓性炎症为特征的自身免疫性肝病,早期即可出现胆管上皮细胞(biliary epithelial cells, BEC)周围炎性细胞浸润. BEC衬覆于胆管内,表达多种病原体识别抗体,并能激活细胞内信号通路,参与免疫调节. PBC病因及发病机制至今尚不完全清楚,但关键步骤是靶向破坏胆管细胞.因胆管上皮细胞参与一系列肝内免疫调节,故胆管上皮细胞损伤是参与PBC肝内炎症发生发展的重要机制,研究胆管上皮细胞BEC损伤的机制,则可为PBC的治疗寻找新靶点.本文就原发性胆汁性胆管炎中胆管上皮细胞损伤的机制研究进展做简要述评.

Primary biliary cholangitis(PBC)is an autoimmune liver disease characterized by chronic biliary cholestasis and progressive intrahepatic and small bile duct nonsuppurative inflammation with early infiltration of inflammatory cells around biliary epithelial cells(BECs).BECs lining the bile duct express multiple receptors for pathogen-associated molecular patterns and can activate intracellular signaling pathways and participate in immune regulation.The etiology and pathogenesis of PBC are not fully understood yet,but the key step found in its pathogenesis is the targeted destruction of biliary cells.Since bile duct epithelial cells participate in a series of intrahepatic immune regulation processes,bile duct epithelial cell injury is an important mechanism involved in the development of intrahepatic inflammation in PBC.Therefore,understanding the mechanism of BEC injury can help us find some new targets for the treatment of PBC.This article briefly reviews the progress in the research of mechanism of biliary epithelial cell injury in PBC.