原发性胆汁性肝硬化发病机制及治疗进展

The Progress of Treatment and Pathogenesis of Primary Biliary Cirrhosis

原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)被认为是自身免疫性肝脏疾病。PBC病因不明,发病机制与遗传因素、环境因素、靶抗原独立活性、T-细胞的自身反应性免疫应答等有关。近来不断有新的物质被鉴定与PBC发病有关。抗线粒体抗体(AMA)为PBC所特有,尤其是AMA-M2高度阳性为PBC的血清学诊断标志。目前治疗尚无法做到对因治疗,以对症和支持治疗为主。熊去氧胆酸(UDCA)是目前唯一被公认对PBC具有特定疗效的药物。目前认为肝移植可提高终末期PBC患者的生存率,延长其生存时间。

Primary biliary cirrhosis （PBC） is considered to be an autoimmune liver disease. Its etiology is unknown. Pathogenesis of PBC is associated with many aspects such as genetic factors, environmental factors, independent activity of target antigen, T-cell autoreactive immune responses and so on. Recently, new materials that involved in the morbidity of PBC have been identified constantly. Antimitochondrial antibodies （AMA） are characteristic of PBC. In particular, AMA-M2 is highly positive for the serological diagnosis mark of PBC. At present, treatment still cannot be for the etiology of PBC. Symptomatic treatment and support treatment are main measures. Ursodeoxycholie acid （UDCA）is the only one recognized medication with specific effect on the PBC. At present, the liver transplant can increase the survival rate and extend their survival time for end-stage of patients with PBC. This article emphasis on the progress of pathogenesis and treatment of the PBC are reviewed to explore research.