Buechter Matthias, Dorn Dominik, Möhlendick Birte, Siffert Winfried, Baba Hideo A, Gerken Guido, Kahraman Alisan
Department of Gastroenterology and Hepatology, University Clinic of Essen, University of Duisburg-Essen, 45122 Essen, Germany.
Department of Gastroenterology and Hepatology, Elisabeth Hospital, 58638 Iserlohn, Germany.
J Clin Med. 2023 Jun 21;12(13):4192. doi: 10.3390/jcm12134192.
Autoimmune hepatitis (AIH) is a complex and progressive inflammatory liver disease characterized by immune-mediated destruction of the liver parenchyma, hypergammaglobulinemia, the presence of circulating autoantibodies, and good response to immunosuppressive therapy. Since the prevalence of AIH is relatively rare, data on the clinical course and the long-term outcome are scarce. We retrospectively analyzed the data of 535 well-documented AIH patients treated at the University Hospital Essen between 2000 and 2020. The majority of patients were middle-aged females (75% women, mean age 45 years) with AIH type 1 (97%). Approximately 32% of patients were diagnosed with cirrhosis due to AIH, 29% had concomitant autoimmune (predominantly autoimmune thyroiditis), and 10% had psychiatric diseases, respectively. Skin tumors were the most common malignant diseases (47% of all tumors), while hepatocellular carcinoma rarely occurred (only six cases). Overall long-term mortality and liver-associated mortality were 9.16% and 4.67%, respectively. However, long-term survival was strongly associated with disease remission. Although AIH is a silent disease and cirrhosis is present in many cases, a favorable long-term prognosis can be achieved by consequent immunosuppressive therapy. The incidence of (liver-associated) complications seems to be lower in comparison to other etiologies, such as viral hepatitis or NASH, and mainly depends on the long-term side effects of immunosuppressive therapy.
自身免疫性肝炎（AIH）是一种复杂的进行性炎症性肝病，其特征为免疫介导的肝实质破坏、高球蛋白血症、循环自身抗体的存在以及对免疫抑制治疗反应良好。由于AIH的患病率相对较低，关于其临床病程和长期预后的数据较少。我们回顾性分析了2000年至2020年间在埃森大学医院接受治疗的535例记录完整的AIH患者的数据。大多数患者为中年女性（75%为女性，平均年龄45岁），患1型AIH（97%）。分别约有32%的患者因AIH被诊断为肝硬化，29%伴有自身免疫性疾病（主要是自身免疫性甲状腺炎），10%患有精神疾病。皮肤肿瘤是最常见的恶性疾病（占所有肿瘤的47%），而肝细胞癌很少发生（仅6例）。总体长期死亡率和肝脏相关死亡率分别为9.16%和4.67%。然而，长期生存与疾病缓解密切相关。尽管AIH是一种隐匿性疾病且许多病例存在肝硬化，但通过持续的免疫抑制治疗可获得良好的长期预后。与其他病因（如病毒性肝炎或非酒精性脂肪性肝炎）相比，（肝脏相关）并发症的发生率似乎较低，且主要取决于免疫抑制治疗的长期副作用。
