Francque Sven, Vonghia Luisa, Ramon Albert, Michielsen Peter
Antwerp University Hospital, Department of Gastroenterology Hepatology, Antwerp, Belgium ; Antwerp University, Faculty of Medicine and Health Sciences, Laboratory of Experimental Medicine and Paediatrics, Antwerp, Belgium.
Antwerp University Hospital, Department of Gastroenterology Hepatology, Antwerp, Belgium ; Department of Internal Medicine, Immunology and Infectious Diseases, University of Bari, Italy.
Hepat Med. 2012 Mar 16;4:1-10. doi: 10.2147/HMER.S16321.
Autoimmune hepatitis (AIH) is a chronic inflammatory disease of the liver that occurs worldwide with a low and probably underestimated prevalence. Although it typically affects young and middle-aged women, it can occur in both sexes and across all age groups. AIH runs a fluctuating course, but can present as severe and even fulminant hepatic failure or at a stage of advanced fibrosis or cirrhosis. Prognosis of severe AIH is poor if untreated. The pathogenesis is complex, combining environmental factors (external chemical or infectious triggers) and host genetic susceptibility. The diagnosis is based, after exclusion of other etiologies of chronic liver disease, on a combination of different elements, including the presence of elevated transaminases, elevated immunoglobulin G (IgG) levels, the presence and pattern of typical autoantibodies, and a liver biopsy showing interface hepatitis and other characteristic features. No single test can be used to make the diagnosis. Response to treatment can also help to establish the diagnosis. Simplified criteria can be used to make a bedside diagnosis with relatively high accuracy. Treatment consists of corticosteroids or other immunosuppressive regimens according to the severity of the disease, the response to the treatment, and the tolerance to therapy, with liver transplantation as an ultimate remedy in treatment-resistant cases with liver decompensation.
自身免疫性肝炎（AIH）是一种肝脏慢性炎症性疾病，在全球范围内均有发生，其患病率较低且可能被低估。尽管它通常影响年轻和中年女性，但男女均可发病，且各年龄组都有出现。AIH病情呈波动变化，但可表现为严重甚至暴发性肝衰竭，或处于晚期纤维化或肝硬化阶段。未经治疗的重度AIH预后较差。其发病机制复杂，涉及环境因素（外部化学或感染诱因）和宿主遗传易感性。诊断需在排除其他慢性肝病病因后，依据多种因素综合判断，包括转氨酶升高、免疫球蛋白G（IgG）水平升高、典型自身抗体的存在及其模式，以及肝活检显示界面性肝炎和其他特征性表现。没有单一的检查可用于确诊，对治疗的反应也有助于诊断。简化标准可用于在床边做出相对准确的诊断。治疗根据疾病严重程度、对治疗的反应及治疗耐受性，采用皮质类固醇或其他免疫抑制方案，对于治疗抵抗且出现肝失代偿的病例，肝移植是最终的治疗手段。
