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自身免疫性肝炎。

Autoimmune hepatitis.

机构信息

Institute of Liver Studies, King's College Hospital NHS Foundation Trust, Denmark Hill, London, UK.

出版信息

Lancet. 2013 Oct 26;382(9902):1433-44. doi: 10.1016/S0140-6736(12)62163-1. Epub 2013 Jun 14.

Abstract

Autoimmune hepatitis is a disease of the hepatic parenchyma that can present in acute or chronic forms. In common with many autoimmune diseases, autoimmune hepatitis is associated with non-organ-specific antibodies in the context of hepatic autoimmunity. This dichotomy has made definition of a unifying hypothesis in the pathophysiology of the disease difficult, although data from the past 8 years have drawn attention to the role of regulatory T cells. Several triggers have been identified, and the disease arises in genetically susceptible individuals. Clinical and biochemical remission is achievable in up to 85% of cases. For the remaining patients, alternative immunosuppression strategies are an option. Liver transplantation provides an excellent outcome for patients with acute liver failure or complications of end-stage liver disease, including hepatocellular carcinoma. Variant or overlapping syndromes are worthy of consideration when unexpected disease features arise.

摘要

自身免疫性肝炎是一种肝实质疾病,可呈急性或慢性形式。与许多自身免疫性疾病一样,自身免疫性肝炎与肝自身免疫中的非器官特异性抗体有关。这种二分法使得难以确定疾病病理生理学的统一假说,尽管过去 8 年的数据引起了对调节性 T 细胞作用的关注。已经确定了几种触发因素,并且该疾病发生在遗传易感个体中。多达 85%的病例可实现临床和生化缓解。对于其余患者,可选择替代免疫抑制策略。对于急性肝衰竭或终末期肝病并发症(包括肝细胞癌)的患者,肝移植提供了极好的结果。当出现意外的疾病特征时,值得考虑变体或重叠综合征。

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