摘要
Primary biliary cirrhosis （PBC） is a disease of unknown etiology leading to progressive destruction of small intrahepatic bile ducts and eventually to liver cirrhosis and failure. It is characterised by female predominance and serum auto-antibodies to mitochondrial antigens targeting the E2 components of the 2-oxoacid dehydrogenase complex. Although they are associated with disease pathogenesis, no concrete evidence has been presented so far. Epidemiological data indicate that a geographical clustering of cases and possible environmental factors are implicated in pathogenesis. A number of genetic factors play a role in determining disease susceptibility or progression, although no definitive conclusion has been reached so far. A key factor to immune pathogenesis is considered to be the breakdown of immune tolerance, either through molecular mimicry or through the so called determinant density model. In this review, the available data regarding the pathogenesis of primary biliary cirrhosis are described and discussed. A new unifying hypothesis based on early endothelin overproduction in primary biliary cirrhosis （PBC） is presented and discussed.
主要胆汁性肝硬变(PBC ) 是导致小肝内胆汁管并且最后的进步破坏到肝肝硬化和失败的未知病原学的疾病。它被女优势和浆液自身抗体描述到指向 2-oxoacid 脱氢酶建筑群的 E2 部件的 mitochondrial 抗原。尽管他们与疾病致病被联系，没有具体证据到目前为止被介绍了。流行病学的数据显示盒子和可能的环境因素的一个地理聚类在致病被含有。很多个基因因素在决定疾病危险性或前进起一个作用，尽管没有权威的结论到目前为止被得出了。到有免疫力的致病的一个关键因素被认为是有免疫力的忍耐的故障，通过分子的模仿或通过所谓的决定因素，密度当模特儿。在这评论，关于主要胆汁性肝硬变的致病的可得到的数据被描述并且讨论。一个新统一假设基于在主要胆汁性肝硬变(PBC ) 的早 endothelin 生产过剩被介绍并且讨论。
