Yokomori Hiroaki, Obu Makoto, Uematsu Takayuki, Okada Takemichi, Yamazaki Hitoshi, Oda Masaya
Department of Internal Medicine.
Division of Pathology, Kitasato University Medical Centre.
Medicine (Baltimore). 2018 Dec;97(52):e13873. doi: 10.1097/MD.0000000000013873.
Autoimmune hepatitis (AIH) is characterised by interface hepatitis. However, some acute cases exhibit atypical centrilobular necrosis with mild portal inflammation. Detailed histological and ultrastructural analyses of acute AIH are limited.
A 44-year-old female was admitted to our hospital with jaundice, general fatigue and liver dysfunction. Her transaminase levels were elevated, her immunoglobulin G level was 1735 mg/dL and her anti-nuclear titres were ×80.
AIH was diagnosed, and histochemical examination of a liver biopsy showed the presence of atypical histological features of prominent centrilobular necrosis and central vein and hepatic sinusoidal endotheliitis. Electron microscopy showed that dendritic cells (DCs) and lymphocytes were attached to disrupted liver sinusoidal endothelial cells (LSECs) in hepatic sinusoids and that DCs attached to LSECs via pseudopods in the central vein.
The patient was started on 40 mg/day prednisolone to control the hepatic inflammation. Her aspartate and alanine aminotransferase levels started declining after prednisolone was initiated. Three weeks later, these levels had normalised. The dosage of prednisolone was gradually decreased as liver function improved. The patient remains under observation and continues to receive 2.5 mg prednisolone.
An important marker of acute AIH may be the presence of activated DCs in the hepatic sinusoids and central vein.
自身免疫性肝炎（AIH）的特征为界面性肝炎。然而，一些急性病例表现出非典型的小叶中心坏死伴轻度门管区炎症。急性AIH的详细组织学和超微结构分析有限。
一名44岁女性因黄疸、全身乏力和肝功能障碍入院。她的转氨酶水平升高，免疫球蛋白G水平为1735mg/dL，抗核抗体滴度为×80。
诊断为AIH，肝活检的组织化学检查显示存在突出的小叶中心坏死以及中央静脉和肝血窦内皮炎等非典型组织学特征。电子显微镜检查显示，树突状细胞（DCs）和淋巴细胞附着于肝血窦中破坏的肝血窦内皮细胞（LSECs），并且DCs通过中央静脉中的伪足附着于LSECs。
患者开始服用40mg/天的泼尼松龙以控制肝脏炎症。开始使用泼尼松龙后，她的天冬氨酸和丙氨酸转氨酶水平开始下降。三周后，这些水平恢复正常。随着肝功能改善，泼尼松龙的剂量逐渐减少。患者仍在观察中，继续服用2.5mg泼尼松龙。
急性AIH的一个重要标志物可能是肝血窦和中央静脉中存在活化的DCs。
