自身免疫性肝炎(AIH)是一种慢性、复发和缓解，免疫介导的肝病，如果不及时治疗，进展为肝硬化。大量患者可能出现急性肝炎或急性肝衰竭，常被误诊为中毒性肝损伤。AIH在年轻女性中占优势，但在儿童和老人中也可以看到。诊断需要整合临床，生物化学，和血清学参数，以及支持性肝组织学和排除其他肝病原因。肝活检是诊断AIH的先决条件，评估疾病的严重程度和阶段，排除其他实体，并识别任何并发的疾病。没有单一的生物标志物或组织学特征是AIH的病理标志。自1993年国际自身免疫性肝炎组织（IAIHG）提出原始评分系统以来，诊断和组织学标准已进行了多次修改。最近,IAIHG提出了组织学标准的共识建议，与急性和慢性AIH相关。这篇综述文章将描述AIH不断发展的诊断标准，由于它们的局限性和实用性，并强调肝组织学在AIH诊断和治疗中的作用。
Autoimmune hepatitis (AIH) is a chronic, relapsing and remitting, immune-mediated liver disease that progresses to cirrhosis if left untreated. A significant number of patients may present with acute hepatitis or acute liver failure, which are often misdiagnosed as toxic liver injury. AIH shows a preponderance in young women but may be seen in children and the elderly. Diagnosis requires the integration of clinical, biochemical, and serologic parameters, along with supportive liver histology and exclusion of other causes of liver disease. Liver biopsy is a prerequisite for diagnosis of AIH, to assess severity and stage of disease, exclude other entities, and recognize any concurrent morbidities. No single biomarker or histologic feature is pathognomonic for AIH. The diagnostic and histologic criteria have undergone several modifications since the original scoring system was proposed by the International Autoimmune Hepatitis Group (IAIHG) in 1993. Recently, the IAIHG has proposed consensus recommendations for histologic criteria, relevant for both acute and chronic AIH. This review article will describe the evolving diagnostic criteria for AIH, with their limitations and utility, and with an emphasis on the role of liver histology in the diagnosis and management of AIH.
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