Araki Toshihiro, Arinaga-Hino Teruko, Koga Hironori, Akiba Jun, Ide Tatsuya, Okabe Yoshinobu, Kuwahara Reiichiro, Amano Keisuke, Yasumoto Makiko, Kawaguchi Toshihiro, Sano Tomoya, Kondou Reiichiro, Kurata Seiji, Mitsuyama Keiichi, Torimura Takuji
Division of Gastroenterology, Department of Internal Medicine, Kurume University School of Medicine, Kurume, Japan.
Division of Gastroenterology and Translational Medicine, Department of Internal Medicine, Kurume University School of Medicine, Kurume, Japan.
Hepatol Res. 2018 Oct;48(11):937-944. doi: 10.1111/hepr.13188. Epub 2018 Jun 4.
Immunoglobulin (Ig)G4-related autoimmune hepatitis (AIH) is a recently proposed subtype that responds well to steroid treatment; however, its pathogenesis remains unclear. We report here a 65-year-old Japanese woman with skin itching and lip swelling. She had liver injury with jaundice, which persisted despite stopping anti-allergic agents. Blood chemistry revealed highly elevated serum IgG and IgG4 (535 mg/dL) levels, and positive anti-nuclear antibody. The diagnosis of AIH was based on liver biopsy. Notably, the IgG4 /IgG cell ratio was 85%. On fluorodeoxyglucose (FDG) positron emission tomography/computed tomography, robust signal intensity was found in the liver, and in enlarged lymph nodes and salivary glands with confirmed IgG4 cell infiltration. Immunofluorescence analysis of the liver biopsy specimen indicated clear expression of glucose transporter-3 (Glut-3) in IgG4 inflammatory cells infiltrating into the portal area. This is the first report of simultaneous strong accumulation of FDG and Glut-3 expression in IgG4-related AIH, which might aid in elucidating the pathogenesis of this disease.
免疫球蛋白（Ig）G4相关性自身免疫性肝炎（AIH）是最近提出的一种亚型，对类固醇治疗反应良好；然而，其发病机制仍不清楚。我们在此报告一名65岁的日本女性，有皮肤瘙痒和嘴唇肿胀症状。她有肝损伤伴黄疸，停用抗过敏药物后仍持续存在。血液化学检查显示血清IgG和IgG4（535mg/dL）水平高度升高，抗核抗体呈阳性。AIH的诊断基于肝活检。值得注意的是，IgG4/I g细胞比率为85%。在氟脱氧葡萄糖（FDG）正电子发射断层扫描/计算机断层扫描中，在肝脏、肿大的淋巴结和唾液腺中发现了强烈的信号强度，证实有IgG4细胞浸润。肝活检标本的免疫荧光分析表明，浸润到门脉区的IgG4炎性细胞中葡萄糖转运蛋白-3（Glut-3）表达明显。这是关于FDG在IgG4相关性AIH中同时强烈积聚和Glut-3表达的首次报告，这可能有助于阐明该疾病的发病机制。
