Primary biliary cirrhosis (PBC)is a chronic autoimmune cholestatic liver disease that manifests a latitudinal gradient in prevalence and incidence. The mechanisms leading to the initiation and perpetuation of PBC remain largely enigmatic, although it is established that a combination of genetic predisposition and environmental stimulation is required. PBC is also characterized by a high concordance rate in monozygotic twins and is considered a model autoimmune disease because of several features common to other conditions and the relatively homogeneous serological and biochemical features. From a diagnostic standpoint, PBC is characterized by the highest specificity of serum autoantibodies directed at mitochondrial proteins. Several risk factors have been suggested to be associated with PBC, including exposure to infectious agents and chemical xenobiotics that will be critically discussed in the present review article.

中文翻译：

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原发性胆汁性肝硬化病因学中的病原体和异生素。

原发性胆汁性肝硬化 (PBC) 是一种慢性自身免疫性胆汁淤积性肝病，其患病率和发病率呈纬度梯度。导致 PBC 发生和延续的机制在很大程度上仍然是个谜，尽管已经确定需要遗传易感性和环境刺激的结合。PBC 的另一个特点是在同卵双胞胎中具有高一致性，并且被认为是一种模型自身免疫性疾病，因为它具有与其他条件相同的几个特征以及相对同质的血清学和生化特征。从诊断的角度来看，PBC 的特点是针对线粒体蛋白的血清自身抗体具有最高的特异性。一些风险因素被认为与 PBC 相关，