Ohno Yoshinori, Kumagi Teru, Imamura Yoshiki, Kuroda Taira, Koizumi Mitsuhito, Watanabe Takao, Yoshida Osamu, Tokumoto Yoshio, Takeshita Eiji, Abe Masanori, Harada Kenichi, Hiasa Yoichi
Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime, 791-0295, Japan.
Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Ishikawa, 920-8640, Japan.
Clin J Gastroenterol. 2018 Feb;11(1):62-68. doi: 10.1007/s12328-017-0787-3. Epub 2017 Nov 1.
Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is often associated with type 1 autoimmune pancreatitis, and the frequency of isolated IgG4-SC seems to be quite low, making the diagnosis of isolated IgG4-SC challenging. A 63-year-old male was admitted to our hospital for frequent fever. Abdominal magnetic resonance cholangiopancreatography showed diffuse narrowing of the common bile duct and post-stenotic dilatation of the right posterior bile duct. Laboratory tests showed abnormalities in the levels of hepatobiliary enzymes and serum IgG4 levels. Endoscopic retrograde cholangiopancreatography showed diffuse narrowing of intrahepatic bile ducts and post-stenotic dilatation of the right posterior bile duct but no abnormalities in the pancreas. Intraductal ultrasonography showed symmetric circumferentially thickened walls of both narrowed and non-narrowed common bile ducts. Histologic examination of the common bile duct mucosa showed infiltration of IgG4-positive plasma cells. Laparoscopic observations showed discoloration with red lobular markings and multiple small depressed lesions. Liver histology showed mild cholangitis with infiltration of IgG4-positive plasma cells around the bile ducts. From these findings, the patient was diagnosed with isolated IgG4-SC. After treatment with a steroid, bile duct dilatations improved. Laparoscopy and intraductal ultrasonography were useful to diagnose isolated IgG4-SC.
免疫球蛋白G4相关性硬化性胆管炎（IgG4-SC）常与1型自身免疫性胰腺炎相关，而孤立性IgG4-SC的发生率似乎相当低，这使得孤立性IgG4-SC的诊断具有挑战性。一名63岁男性因反复发热入住我院。腹部磁共振胰胆管造影显示胆总管弥漫性狭窄及右后胆管狭窄后扩张。实验室检查显示肝胆酶水平及血清IgG4水平异常。内镜逆行胰胆管造影显示肝内胆管弥漫性狭窄及右后胆管狭窄后扩张，但胰腺无异常。胆管内超声显示狭窄和未狭窄的胆总管壁均呈对称的环形增厚。胆总管黏膜组织学检查显示IgG4阳性浆细胞浸润。腹腔镜观察显示有红色小叶状斑纹的变色及多个小凹陷性病变。肝脏组织学显示轻度胆管炎，胆管周围有IgG4阳性浆细胞浸润。根据这些发现，该患者被诊断为孤立性IgG4-SC。经类固醇治疗后，胆管扩张有所改善。腹腔镜检查和胆管内超声对孤立性IgG4-SC的诊断很有用。
