原发性胆汁性肝硬化(primarybiliarycirrhosis,PBC)是一种累及肝内胆管系统的慢性进展性疾病,多见于中年女性,肝脏组织病理特点为慢性进行性、非化脓性、破坏性小胆管炎,表现为肝内小叶间胆管炎症、肉芽肿形成、小胆管内胆汁淤积、胆管缺失等,最终导致肝细胞破坏、肝纤维化、肝 Primary biliary cirrhosis (primarybiliarycirrhosis, PBC) is a chronic progressive disease involving the intrahepatic bile duct system, more common in middle-aged women, liver histopathological features of chronic progressive, non purulent, destructive cholangitis , Manifested as intrahepatic interlobular biliary inflammation, granuloma formation, small bile duct cholestasis, bile duct loss, eventually leading to the destruction of liver cells, liver fibrosis, liver