Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the main autoimmune cholestatic liver diseases. IgG4-associated sclerosing cholangitis is another distinct immune-mediated cholestatic disorder of unknown aetiology that is frequently associated with autoimmune pancreatitis or other IgG4-related diseases. Although the majority of PBC and PSC patients have a typical presentation, there are common and uncommon important variants or specific subgroups that observed in everyday routine clinical practice. In this updated review, we summarize the published data giving also our own experience on the variants and specific groups of autoimmune cholestatic liver diseases. Actually, we give in detail the underlining difficulties and the rising dilemmas concerning the diagnosis and management of these special conditions in the clinical spectrum of autoimmune cholestatic liver diseases including the IgG4-associated sclerosing cholangitis highlighting also the uncertainties and the potential new eras of the research agenda.

中文翻译：

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自身免疫性胆汁淤积性肝病的变异形式和特定形式。

原发性胆汁性胆管炎（PBC）和原发性硬化性胆管炎（PSC）是主要的自身免疫性胆汁淤积性肝病。IgG4相关性硬化性胆管炎是另一种病因不明的免疫介导的胆汁淤积性疾病，通常与自身免疫性胰腺炎或其他IgG4相关疾病有关。尽管大多数PBC和PSC患者都有典型的表现，但在日常的常规临床实践中仍观察到常见且不常见的重要变异或特定亚组。在本更新的综述中，我们总结了已发表的数据，还提供了我们自身免疫性胆汁淤积性肝病变体和特定人群的经验。其实，