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摘要:
自身免疫性肝炎（AIH）是免疫介导的肝实质炎症性疾病，以高丙种球蛋白血症、自身抗体阳性和典型的肝组织学异常为特征。然而，AIH临床表现多样，缺乏特异性的血清学标志物，因此AIH的诊断困难，具有挑战性。虽然汇管区淋巴浆细胞浸润、界面性肝炎、淋巴细胞穿入现象和肝细胞玫瑰花结为AIH典型的组织学特征，但在AIH中尚可观察到其他的一些形态学改变，包括小叶中心性坏死、Kupffer细胞内透明小球等。所以没有一个单一的组织学特征可直接诊断AIH，需结合临床和实验室检查，并排除其他原因引起的肝脏疾病，方可做出正确的诊断。本文总结了AIH的组织学特征、不同的病理组织学谱、常见的临床问题、主要的鉴别诊断和最新进展。
Abstract:Autoimmune hepatitis （AIH） is an immune-mediated inflammatory disease of the liver parenchyma， which is characterized by hypergammaglobulinemia， the presence of autoantibodies， and typical abnormalities in liver histology； however， the diverse clinical manifestations of AIH and the lack of specific serological markers have brought difficulties and challenges in the diagnosis of AIH. Although portal lymphoplasmacytic infiltration， interface hepatitis， lymphocyte emperipolesis， and hepatocyte rosettes are the typical histological features of AIH， many other histological features can also be observed in AIH， including centrilobular necrosis and Kupffer cell hyaline globules. Therefore， no single histological feature can be used for the diagnosis of AIH， and a confirmed diagnosis should be made with reference to clinical and laboratory examinations， with the exclusion of liver diseases due to other causes. This article summarizes the histological features of AIH， different histopathological spectrum， common clinical issues， differential diagnosis， and recent advances.
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Key words:
- Hepatitis， Autoimmune /
- Biopsy /
- Pathology /
- Diagnosis
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注： a，汇管区淋巴浆细胞浸润，界面性肝炎（HE染色，×10）b，汇管区簇状浆细胞浸润（箭头）（HE染色，×40）；c，簇状浆细胞阳性（箭头）（MUM-1染色，×20）；d，簇状浆细胞阳性（箭头）（CD38染色，×20）。
图 1 汇管区淋巴浆细胞浸润和界面性肝炎
Figure 1. Portal lymphoplasmacytic infiltrate and interface hepatitis
注： 箭头处为小叶中心性坏死。
图 4 小叶中心性坏死（HE染色，×10）
Figure 4. Centrilobular necrosis（HE staining，×10）
注： 箭头处为合胞体多核巨肝细胞。
图 5 合胞体多核巨肝细胞（HE染色，×20）
Figure 5. Syncytial multinucleated giant cells（HE staining，×20）
表 1 基于汇管区和小叶炎症的AIH诊断标准
Table 1. AIH diagnostic criteria based on portal area and lobular inflammation
诊断 汇管区炎症 小叶炎症 极可能 （likely AIH） 汇管区淋巴、浆细胞浸润+ 以下1种或2种特征： （1）轻度以上界面性肝炎， （2）轻度以上小叶炎； 缺乏提示其他肝病的组织学形态 轻度以上小叶炎（伴或不伴小叶中心性坏死）+至少1种以下特征： （1）淋巴、浆细胞浸润， （2）界面性肝炎， （3）汇管区纤维化； 缺乏提示其他肝病的组织学形态 可能 （possible AIH） 汇管区淋巴、浆细胞浸润； 不具有上述极可能的任一特征； 缺乏提示其他肝病的组织学形态或 伴上述1种或2种极可能的特征； 出现提示其他肝病的组织学形态 小叶性肝炎，伴或不伴小叶中心性坏死； 不具有上述极可能的任一特征； 缺乏提示其他肝病的组织学形态或 伴上述任一极可能的特征； 出现提示其他肝病的组织学形态 不太可能 （likely AIH） 汇管区炎症； 不具有上述极可能的任一特征； 出现提示其他肝病的组织学形态 任何程度的小叶性肝炎； 不具有上述极可能的任一特征； 出现提示其他肝病的组织学形态 
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