Sawai Hiroaki, Matsubayashi Hiroyuki, Sasaki Keiko, Tanaka Masaki, Kakushima Naomi, Takizawa Kohei, Yamaguchi Yuichiro, Ono Hiroyuki
Division of Endoscopy, Shizuoka Cancer Center, Japan.
Intern Med. 2011;50(5):433-8. doi: 10.2169/internalmedicine.50.4471. Epub 2011 Mar 1.
Sclerosing cholangitis (SC) is one of the lesions frequently seen in IgG4-related systemic diseases, causing biliary stricture and mimicking bile duct carcinoma and primary sclerosing cholangitis (PSC). Although it often accompanies autoimmune pancreatitis (AIP), autoimmune-related SC without a pancreatic lesion is very rare. A 79-year-old woman was referred to our institution with suspected diagnosis of bile duct carcinoma in the previous hospital. The patient was not icteric and fever free, but with an elevated level of serum biliary enzyme, which lead us to detect this disease. Clinical images including computed tomography (CT), endoscopic retrograde cholangiopancreatography (ERCP) and intraductal ultrasonography (IDUS) demonstrated multiple strictures at the intrahepatic bile duct and enhanced wall thickness at the upper common bile duct, however her pancreas was normal. Repeated endoscopic procedures with multiple biopsies from the biliary strictures demonstrated fibrous ductal tissues with lymph-plasma cell infiltration (>10 IgG4(+) cells/HPF). By positron emission tomography using (18)F-fluorodeoxyglucose (FDG-PET), the uptake of FDG was not remarkable in areas other than the biliary lesions. Additional laboratory tests showed elevated levels of serum IgG (2,571 mg/dL), and γ-globulin (29%), and positive autoantibodies, but normal IgG4 (53.2 mg/dL). Together with clinical images, laboratory and histological findings, we diagnosed this patient as sclerosing cholangitis which was thought to be associated with autoimmunity. After one year of follow-up without steroid therapy, idiopathic thrombocytopenic purpura (ITP) developed with an increased level of serological markers. We encountered a rare case of sclerosing cholangitis expected to be associated with autoimmunity, which showed biliary strictures mimicking bile duct carcinoma and needed careful diagnosis. Unlike the typical AIP, the current case demonstrated distinct serological findings and no other organ involvement. Further study is needed to clarify the characteristics of sclerosing cholangitis associated with autoimmunity with a large number of cases.
硬化性胆管炎（SC）是IgG4相关系统性疾病中常见的病变之一，可导致胆管狭窄，并酷似胆管癌和原发性硬化性胆管炎（PSC）。虽然它常伴有自身免疫性胰腺炎（AIP），但无胰腺病变的自身免疫相关性SC非常罕见。一名79岁女性因前一家医院怀疑诊断为胆管癌而转诊至我院。患者无黄疸且无发热，但血清胆汁酶水平升高，这促使我们发现了该疾病。包括计算机断层扫描（CT）、内镜逆行胰胆管造影（ERCP）和导管内超声检查（IDUS）在内的临床影像显示肝内胆管多处狭窄，肝总管上段壁增厚，但胰腺正常。对胆管狭窄部位进行多次活检的反复内镜检查显示，纤维性导管组织有淋巴浆细胞浸润（>10个IgG4(+)细胞/高倍视野）。通过使用（18）F-氟脱氧葡萄糖（FDG-PET）的正电子发射断层扫描，除胆管病变外，其他区域的FDG摄取并不显著。进一步的实验室检查显示血清IgG（2571mg/dL）、γ-球蛋白（29%）水平升高，自身抗体阳性，但IgG4正常（53.2mg/dL）。结合临床影像、实验室和组织学检查结果，我们将该患者诊断为与自身免疫相关的硬化性胆管炎。在未接受类固醇治疗的一年随访后，出现了特发性血小板减少性紫癜（ITP），血清学标志物水平升高。我们遇到了一例罕见的预计与自身免疫相关的硬化性胆管炎病例，该病例表现为酷似胆管癌的胆管狭窄，需要仔细诊断。与典型的AIP不同，本例表现出独特的血清学检查结果，且无其他器官受累。需要进一步研究以通过大量病例阐明与自身免疫相关的硬化性胆管炎的特征。
