Taubert Richard, Engel Bastian
Klinik für Gastroenterologie, Hepatologie, Infektiologie und Endokrinologie, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland.
Inn Med (Heidelb). 2024 Apr;65(4):325-333. doi: 10.1007/s00108-024-01679-2. Epub 2024 Mar 8.
Autoimmune hepatitis (AIH) is a rare autoimmune inflammation of the liver mostly with a chronic course, which can also be acutely manifested up to acute liver failure. It affects women 3-4 times more frequently than men and can be diagnosed in all age groups. In one third of the patients a liver cirrhosis is present at the time of diagnosis. It is characterized by a hepatic inflammation pattern, a polyclonal hypergammaglobulinemia of immunoglobulin G and the detection of autoantibodies. A liver biopsy is necessary to make the diagnosis. The AIH is histologically characterized in particular by a lymphoplasmacytic infiltrate in the portal fields. In cases with a relevant disease activity, AIH is typically treated by immunosuppression. The immunosuppressive treatment is associated with a prevention of disease progression to liver cirrhosis and a better survival. The success of treatment is measured by achieving biochemical remission, i.e., normalization of the transaminase and immunoglobulin G levels as a good noninvasive predictor of a histological remission. Another treatment target is an improvement of the symptoms of the patient. The first-line treatment consists of a glucocorticoid, mostly prednisolone or in cases without advanced fibrosis budesonide, and azothioprine. For reduction of steroid-specific treatment side effects the maintenance treatment should be carried out steroid-free whenever possible. In cases of insufficient response to azothioprine or side effects a treatment attempt using antimetabolites, such as 6‑mercaptopurine or mycophenolate mofetil is primarily carried out as second-line treatment. For patients who do not achieve biochemical remission through first-line or second-line treatment, a variety of medications are available for third-line treatment, e.g., rituximab, calcineurin inhibitors or antitumor necrosis factor (anti-TNF) antibodies. Third-line treatment should be carried out in expert centers and registered in the European Reference Network for Rare Liver Diseases in order to improve the currently sparse database for these forms of treatment in the future.
自身免疫性肝炎（AIH）是一种罕见的肝脏自身免疫性炎症，大多病程呈慢性，也可急性发作直至急性肝衰竭。女性受其影响的频率比男性高3至4倍，各年龄组均可诊断出该病。三分之一的患者在诊断时已出现肝硬化。其特征为肝脏炎症模式、免疫球蛋白G的多克隆高球蛋白血症以及自身抗体的检测。肝活检是确诊所必需的。AIH在组织学上的特征尤其表现为门管区淋巴细胞和浆细胞浸润。在疾病活动度较高的情况下，AIH通常采用免疫抑制治疗。免疫抑制治疗可预防疾病进展为肝硬化，并提高生存率。治疗成功的衡量标准是实现生化缓解，即转氨酶和免疫球蛋白G水平正常化，这是组织学缓解的良好非侵入性预测指标。另一个治疗目标是改善患者症状。一线治疗包括糖皮质激素，大多为泼尼松龙，对于无晚期纤维化的病例则使用布地奈德，以及硫唑嘌呤。为减少类固醇特异性治疗副作用，维持治疗应尽可能不使用类固醇。在对硫唑嘌呤反应不足或出现副作用的情况下，主要采用抗代谢药物如6-巯基嘌呤或霉酚酸酯进行二线治疗尝试。对于通过一线或二线治疗未实现生化缓解的患者，有多种药物可用于三线治疗，例如利妥昔单抗、钙调神经磷酸酶抑制剂或抗肿瘤坏死因子（抗TNF）抗体。三线治疗应在专家中心进行，并在欧洲罕见肝病参考网络中登记，以便未来完善目前关于这些治疗方式的稀疏数据库。
