Muratori Luigi, Lohse Ansgar W, Lenzi Marco
DIMEC, Università di Bologna and IRCCS Policlinico di Sant'Orsola, Bologna, Italy
European Reference Network for Hepatological Diseases (ERN RARE-LIVER).
BMJ. 2023 Feb 6;380:e070201. doi: 10.1136/bmj-2022-070201.
Autoimmune hepatitis is an inflammatory disease of the liver of unknown cause that may progress to liver cirrhosis and end stage liver failure if diagnosis is overlooked and treatment delayed. The clinical presentation is often that of acute hepatitis, sometimes very severe; less frequently, it can be insidious or completely asymptomatic. The disease can affect people of any age and is more common in women; its incidence and prevalence seem to be on the rise worldwide. An abnormal immune response targeting liver autoantigens and inducing persistent and self-perpetuating liver inflammation is the pathogenic mechanism of the disease. A specific set of autoantibodies, increased IgG concentrations, and histological demonstration of interface hepatitis and periportal necrosis are the diagnostic hallmarks of autoimmune hepatitis. Prompt response to treatment with corticosteroids and other immunomodulatory drugs is almost universal and supports the diagnosis. The aims of treatment are to induce and maintain long term remission of liver inflammation. Treatment can often even reverse liver fibrosis, thus preventing progression to advanced cirrhosis and its complications. Most patients need lifelong maintenance therapy, and repeated follow-up in experienced hands improves the quality of care and quality of life for affected patients.
自身免疫性肝炎是一种病因不明的肝脏炎症性疾病，如果诊断被忽视且治疗延迟，可能会进展为肝硬化和终末期肝衰竭。其临床表现通常为急性肝炎，有时非常严重；较少见的情况下，病情可能隐匿或完全无症状。该疾病可影响任何年龄段的人群，在女性中更为常见；其发病率和患病率在全球范围内似乎都在上升。针对肝脏自身抗原的异常免疫反应并诱导持续的、自我延续的肝脏炎症是该疾病的致病机制。一组特定的自身抗体、IgG浓度升高以及界面性肝炎和汇管区周围坏死的组织学表现是自身免疫性肝炎的诊断标志。对皮质类固醇和其他免疫调节药物治疗的迅速反应几乎是普遍现象，这也支持了诊断。治疗的目的是诱导并维持肝脏炎症的长期缓解。治疗通常甚至可以逆转肝纤维化，从而防止进展为晚期肝硬化及其并发症。大多数患者需要终身维持治疗，由经验丰富的医生进行反复随访可提高对受影响患者的护理质量和生活质量。
