原发性胆汁性肝硬化(PBC)是一种原因不明的慢性炎症性和胆汁淤积性肝脏疾病。它以肝脏内进行性小胆管损坏伴有门管区炎症、胆汁淤积和肝纤维化为特点,并可最终进展至肝硬化和肝功能衰竭。该病主要发生在中年女性,常合并肝外器官特异性自身免疫性疾病如自身免疫性甲状腺疾病、干 Primary biliary cirrhosis (PBC) is an unexplained chronic inflammatory and cholestatic liver disease. It is characterized by progressive destruction of small bile ducts in the liver accompanied by portal inflammation, cholestasis and liver fibrosis, and eventually to cirrhosis and liver failure. The disease occurs mainly in middle-aged women, often associated with extrahepatic organ-specific autoimmune diseases such as autoimmune thyroid disease, dry