【摘要】 原发性胆汁性肝硬化（PBC）是一种慢性肝内胆汁淤积性疾病，血清抗线粒体抗体（AMA）是诊断PBC的特异性指标，熊去氧胆酸（UDCA）是惟一经随机对照临床试验证实的治疗PBC安全有效的药物。尽管PBC的发病机制可能与自身免疫有关，但免疫抑制剂的疗效仍未被证实，且药物相关不良反应明显，使其临床应用受到限制。PBC的病情呈进行性，最终可导致肝硬化肝功能衰竭，肝移植是治疗终末期PBC患者惟一有效的方法。更多还原

【Abstract】 Primary biliary cirrhosis(PBC) is a chronic intrahepatic cholestatic disease. Serum antimitochondrial antibody(AMA) is a specific criterion for the diagnosis of PBC. Ursodeoxycholic acid(UDCA) is the only safe and effective treatment of PBC proven by randomized controlled clinical trials. Despite the presumed autoimmune etiology of PBC, a clear benefit from immunosuppressive agents has not been demonstrated and their use is limited by the occurrence of side effects. As the disease is progressive and finally may lead to cirrhosis and liver failure, liver transplantation is the only therapeutic option for end-stage patients.更多还原