Duclos-Vallée J C, Johanet C, Sebagh M, Samuel D, Yamamoto A M
Centre Hépato-Biliaire, EPI 99-41 et UPRES 1596, Hôpital Paul-Brousse, 14, avenue Paul-Vaillant-Couturier, 94804 Villejuif.
Ann Med Interne (Paris). 2001 Oct;152(6):371-82.
Autoimmune hepatitis is characterized by an inflammation of the portal tract with lymphocytes and plasma cells, an hypergammaglobulinemia and a variety of circulating autoantibodies. The presence of smooth muscle antibodies and/or antinuclear antibodies define type 1. Type 2 is characterized by the presence of liver-kidney--microsomal antibodies. Environmental, genetic and infectious factors may explain the autoreactivity of T cells. Different non specific clinical features may be present. Sometimes the presentation may be an acute hepatitis; in the remainder, the disease may not be recognized until liver damage is advanced. Hypergammaglobulinemia and presence of circulating autoantibodies are the key for diagnosis. The association of prednisolone in combination with azathioprine remains the established treatment. If relapse or non response occur, other immunosuppressive therapy such as cyclosporin may be useful. Liver transplantation is reserved for (sub)fulminant forms and end stage liver disease.
自身免疫性肝炎的特征为门管区淋巴细胞和浆细胞浸润、高球蛋白血症以及多种循环自身抗体。平滑肌抗体和/或抗核抗体阳性可诊断为1型。2型的特征为肝肾微粒体抗体阳性。环境、遗传和感染因素可能解释T细胞的自身反应性。患者可能出现不同的非特异性临床特征。有时表现为急性肝炎；在其他情况下，直到肝脏损害进展时疾病才会被发现。高球蛋白血症和循环自身抗体的存在是诊断的关键。泼尼松龙联合硫唑嘌呤仍是既定的治疗方法。如果出现复发或无反应，其他免疫抑制疗法如环孢素可能有效。肝移植适用于（亚）暴发性和终末期肝病。
