A 69-year-old man presented with jaundice and appetite loss. Blood analyses showed elevated aminotransferase levels, hyperbilirubinemia, positivity for antinuclear antibody, elevated immunoglobulin (Ig) G4 levels, and negativity for hepatitis virus markers. Additionally, computed tomography revealed a focal enlargement of the pancreatic body and enhancement of the peripheral bile ducts. Liver biopsy showed interface hepatitis, supporting a clinical diagnosis of autoimmune hepatitis (AIH). Immunohistochemistry revealed that IgG4-bearing plasma cells accounted for more than 60% of the IgG-bearing plasma cells in the portal area. Then, we started oral prednisolone therapy. After tapering, serum transaminase levels became elevated again, and we had to adjust the dose. Azathioprine maintenance therapy was necessary to prevent relapse. We herein report a case of IgG4-hepatopathy with a clinical course similar to that of AIH with acute onset.
Case Rep Gastroenterol 2021;15:720–728

中文翻译：

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免疫球蛋白 G4 肝病伴急性肝炎样发作和明显的小叶中心坏死：与免疫球蛋白 G4 相关疾病相关的肝损伤的临床病理学独特模式

一名 69 岁男性因黄疸和食欲减退就诊。血液分析显示转氨酶水平升高、高胆红素血症、抗核抗体阳性、免疫球蛋白 (Ig) G4 水平升高以及肝炎病毒标志物阴性。此外，计算机断层扫描显示胰体局灶性扩大和外周胆管增强。肝活检显示界面性肝炎，支持自身免疫性肝炎（AIH）的临床诊断。免疫组织化学显示，在汇管区，含 IgG4 的浆细胞占含 IgG 浆细胞的 60% 以上。然后，我们开始口服泼尼松龙治疗。减量后，血清转氨酶水平再次升高，我们不得不调整剂量。硫唑嘌呤维持治疗对于预防复发是必要的。
Case Rep Gastroenterol 2021；15:720–728