非典型的な硬化性胆管炎が疑われた若年発症の限局性胆管狭窄の1例

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  • A rare case of sclerosing cholangitis with a dominant stricture of the bile duct at the porta hepatis

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A 31-year-old Japanese man, who worked in Hong Kong, presented with a 1-month history of jaundice and was admitted to our hospital. Blood tests revealed evidence of obstructive jaundice. Abdominal ultrasonography, computed tomography (CT) , and magnetic resonance cholangiopancreatography (MRCP) showed dilatation of the intrahepatic bile ducts and thickening of the upper portion of the bile duct. Endoscopic retrograde cholangiopancreatography (ERCP) revealed a dominant stricture of the bile duct at the porta hepatis. Bile juice and brush cytology performed during the ERCP revealed no evidence of malignancy. A biopsy of the stricture lesion showed inflammatory cell infiltration and mild fibrosis.<br> There was no evidence of malignancy or infection. Additional blood tests showed no evidence of infection or IgG4-related sclerosing cholangitis. Surgical treatment was selected because of the difficulty in ruling out the possibility of malignancy. Histopathological analysis of the resected specimen of the bile duct stricture showed inflammatory changes, but no malignancy. Although sclerosing cholangitis was considered as the most likely diagnosis, all secondary causes of this condition were excluded on the basis of the patient’s history and examinations. Therefore, establishing the diagnosis was difficult in spite of the detailed examination in this case.

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