【摘要】 自身免疫性肝炎（AIH）是由不明原因免疫异常引起的急性或慢性肝脏炎症性疾病，不分年龄或性别，影响所有种族群体。AIH如果没有得到及时的治疗，可能会发展为肝硬化、肝衰竭，甚至导致死亡。目前一些诊断评分系统和肝活组织病理检查已成为诊断的标准，然而由于疾病表现的高度异质性，AIH诊断仍很有挑战性。大多数患者最初对一线治疗（糖皮质激素与硫唑嘌呤的联合治疗）有应答，然而，应答欠佳和因不良反应引起的不耐受也不少见，需要二线和（或）三线治疗。本文总结阐述诊断困难、一线药物治疗应答欠佳或不耐受的疑难AIH诊断和管理的最新进展，并归纳了目前国内外关于AIH治疗的新方法，为AIH的临床诊疗提供参考。更多还原

【Abstract】 Autoimmune hepatitis(AIH)is an acute or chronic inflammatory disease of liver caused by unclear immune response that affects people from all ethnic groups irrespective of age or sex.If left untreated,AIH will lead to cirrhosis,liver failure,or death.A number of diagnostic scoring systems and histopathological examination of liver biopsies are now the standard for diagnosis.However,due to the high heterogeneity of the disease presentation,AIH diagnosing remains challenging.Most patients initially respond to first-line treatment,which is corticosteroids combined with azathioprine.However,insufficient response and intolerance due to side effects are also common,so some patients requires second-and/or third-line therapies.Here we summarized the latest progress in diagnosis and management of AIH with difficult diagnosis,poor response to first-line drug treatment or intolerance,as well as the new methods of AIH treatment worldwide,to provide reference for the clinical diagnosis and treatment of AIH.更多还原