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Abstract：
The management of autoimmune hepatobiliary disorders remains a challenging and emerging area of investigation. An awareness of cholestatic liver diseases is critical to appropriate recognition and management of these challenging diseases, because patients often present asymptomatically, and diagnosis is limited by the lack of disease-specific markers and diagnostic studies. Furthermore, there is a paucity of treatment options because the pathophysiology underlying autoimmune biliary diseases remains largely unknown. This article discusses the natural history, clinical presentation, diagnosis, and medical and surgical management strategies for three dominant autoimmune biliary diseases: primary biliary cirrhosis, primary sclerosing cholangitis, and immunoglobulin G4-related hepatobiliary disease.
摘要：
自身免疫性肝胆疾病的管理仍然是一个具有挑战性和新兴的研究领域。对胆汁淤积性肝病的认识对于正确识别和管理这些具有挑战性的疾病至关重要。因为患者通常无症状地出现，和诊断由于缺乏疾病特异性标志物和诊断研究而受到限制。此外，由于自身免疫性胆道疾病的病理生理学仍在很大程度上未知，因此治疗方案很少。本文讨论了自然史，临床表现,诊断,以及三种主要自身免疫性胆道疾病的医学和外科管理策略：原发性胆汁性肝硬化，原发性硬化性胆管炎,和免疫球蛋白G4相关的肝胆疾病。
