原发性胆汁性肝硬化病理机制浅谈

A brief discussion on pathogenesis of primary biliary cirrhosis

原发性胆汁性肝硬化(PBC)发病机制未明,研究发现同卵双生共患PBC概率较高,而其家族聚集性,全基因组关联分析(GWAS)结果皆表明了遗传因素的重要性;流行病学调查和动物模型实验则表明生物异源物质和感染性分子可能通过分子模拟在发病中起作用;而如下证据则支持其自身免疫机制:出现高度特异性的血清抗线粒体抗体(AMA)和自身反应性T淋巴细胞;就组织病理学而言,PBC的特征为汇管区炎症和免疫介导的肝内胆管破坏。从遗传因素、环境暴露、自身免疫、肝脏病理对PBC的病理机制进行了总结,指出了免疫失衡的重要性。

The pathogenesis of primary biliary cirrhosis （PBC） is unknown. Researchers have found that monozygotic twins have a higher concordance rate for PBC than dizygotic twins, and the phenomenon of familial aggregation and the results of genome - wide association stud- ies also demonstrate the importance of genetic factors in the pathogenesis of PBC. The epidemiological investigations and animal model exper- iments show that xenobiotics and infectious molecules may play a role in the pathogenesis of PBC by molecular mimicry. The appearance of highly specific serum antimitochondrial antibodies and autoreactive T cells suggests a possible autoimmune pathogenesis of PBC. The his- topathological features of PBC are inflammation in the portal area and immune - mediated intrahepatic bile duct destruction. The pathogenesis of PBC is summarized from the aspects of genetic factors, environmental exposure, autoimmune response, and fiver pathology, and the im- portance of immune imbalance is emphasized.