Sahebjam Farhad, Vierling John M
Departments of Medicine and Surgery, Baylor College of Medicine, Baylor-St. Luke's Medical Center, Houston, TX, 77030, USA.
Front Med. 2015 Jun;9(2):187-219. doi: 10.1007/s11684-015-0386-y. Epub 2015 Mar 6.
Autoimmune hepatitis is a chronic liver disease putatively caused by loss of tolerance to hepatocyte-specific autoantigens. It is characterized by female predilection, elevated aminotransferase levels, autoantibodies, increased γ-globulin or IgG levels and biopsy evidence of interface hepatitis. It is currently divided into types 1 and 2, based on expression of autoantibodies. Autoantigenic epitopes have been identified only for the less frequent type 2. Although autoimmune hepatitis occurs in childhood, this review focuses on disease in adults. In the absence of pathognomonic biomarkers, diagnosis requires consideration of clinical, biochemical, serological and histological features, which have been codified into validated diagnostic scoring systems. Since many features also occur in other chronic liver diseases, these scoring systems aid evaluation of the differential diagnosis. New practice guidelines have redefined criteria for remission to include complete biochemical and histological normalization on immunosuppressive therapy. Immunosuppression is most often successful using prednisone or prednisolone and azathioprine; however, the combination of budesonide and azathioprine for non-cirrhotic patients offers distinct advantages. Patients failing standard immunosuppression are candidates for alternative immunosuppressive regimens, yet none of the options has been studied in a randomized, controlled trial. Overlap syndromes with either primary sclerosing cholangitis or primary biliary cirrhosis occur in a minority. Liver transplantation represents a life-saving option for patients presenting with acute liver failure, severely decompensated cirrhosis or hepatocellular carcinoma. Transplant recipients are at risk for recurrent autoimmune hepatitis in the allograft, and de novo disease may occur in patients transplanted for other indications. Patients transplanted for AIH are also at risk for recurrent or de novo inflammatory bowel disease. Progress in our understanding of the immunopathogenesis should lead to identification of specific diagnostic and prognostic biomarkers and new therapeutic strategies.
自身免疫性肝炎是一种慢性肝病，据推测是由于对肝细胞特异性自身抗原的免疫耐受丧失所致。其特征为女性多见、转氨酶水平升高、自身抗体、γ-球蛋白或IgG水平升高以及界面性肝炎的活检证据。目前根据自身抗体的表达情况分为1型和2型。仅在较少见的2型中确定了自身抗原表位。尽管自身免疫性肝炎可发生于儿童期，但本综述重点关注成人疾病。在缺乏特异性生物标志物的情况下，诊断需要综合考虑临床、生化、血清学和组织学特征，这些特征已被编入经过验证的诊断评分系统。由于许多特征也见于其他慢性肝病，这些评分系统有助于评估鉴别诊断。新的实践指南重新定义了缓解标准，包括免疫抑制治疗时生化和组织学完全恢复正常。免疫抑制最常使用泼尼松或泼尼松龙以及硫唑嘌呤；然而，布地奈德和硫唑嘌呤联合用于非肝硬化患者具有明显优势。标准免疫抑制治疗失败的患者可选择其他免疫抑制方案，但尚无任何一种方案在随机对照试验中进行过研究。少数患者会出现与原发性硬化性胆管炎或原发性胆汁性肝硬化的重叠综合征。肝移植是急性肝衰竭、严重失代偿性肝硬化或肝细胞癌患者的救命选择。移植受者的同种异体移植有复发自身免疫性肝炎的风险，而因其他适应证接受移植的患者可能会发生新发疾病。因自身免疫性肝炎接受移植的患者也有复发或新发炎症性肠病的风险。我们对免疫发病机制认识的进展应能促成特异性诊断和预后生物标志物的发现以及新的治疗策略。
