Shinozaki Hiroharu, Sasakura Yuuichi, Shinozaki Satoshi, Terauchi Toshiaki, Matsui Junichi, Kobayashi Kenji, Lefor Alan Kawarai, Ogata Yoshiro
Department of Surgery, Saiseikai Utsunomiya Hospital, Utsunomiya, Japan.
Shinozaki Medical Clinic, Utsunomiya, Japan.
Case Rep Gastroenterol. 2021 Feb 5;15(1):154-162. doi: 10.1159/000512402. eCollection 2021 Jan-Apr.
Autoimmune pancreatitis (AIP) is characterized by pancreatic manifestations of IgG4-related disease. Malignancies in patients with AIP have been reported, but carcinoma of the bile duct is extremely rare. We report a patient with IgG4-related AIP who developed cholangiocarcinoma after 8 years of steroid treatment. A 76-year-old male presented with fever (37.8°C) due to biliary obstruction and cholangitis. He had been treated with steroids for 8 years to control inflammation due to IgG4-related AIP. During 8 years of treatment, hepatobiliary enzyme levels were well controlled within their normal range, but serum IgG4 levels remained elevated. A computed tomography scan showed intrahepatic bile duct dilatation. Magnetic resonance cholangiopancreatography showed obstructive changes at the junction of the cystic and common ducts. To relieve biliary obstruction, endoscopic bile duct drainage using a nasobiliary tube was performed, and cytology was Class IV. Aorto-caval lymph node enlargement was found at laparotomy, intraoperatively diagnosed as adenocarcinoma, and resection was abandoned. He died 4 months postoperatively. We report a patient with IgG4-related AIP complicated by cholangiocarcinoma which developed after 8 years of steroid treatment. Even if hepatobiliary markers are well controlled, periodic follow-up with imaging studies may facilitate detection of an early cholangiocarcinoma.
自身免疫性胰腺炎（AIP）的特征是IgG4相关疾病的胰腺表现。已有报道AIP患者发生恶性肿瘤，但胆管癌极为罕见。我们报告1例IgG4相关AIP患者，在接受类固醇治疗8年后发生胆管癌。1例76岁男性因胆道梗阻和胆管炎出现发热（37.8°C）。他因IgG4相关AIP接受类固醇治疗8年以控制炎症。在8年治疗期间，肝胆酶水平在正常范围内得到良好控制，但血清IgG4水平仍升高。计算机断层扫描显示肝内胆管扩张。磁共振胰胆管造影显示胆囊管与胆总管交界处有梗阻性改变。为缓解胆道梗阻，采用鼻胆管进行内镜胆管引流，细胞学检查为IV级。剖腹手术时发现主动脉腔静脉淋巴结肿大，术中诊断为腺癌，放弃切除。他术后4个月死亡。我们报告1例IgG4相关AIP并发胆管癌患者，该胆管癌在类固醇治疗8年后发生。即使肝胆标志物得到良好控制，定期进行影像学检查随访可能有助于早期胆管癌的检测。
