Abstract

A 63-year-old male was conducted Corticosteroid Therapy (CST) in the diagnosis of autoimmune pancreatitis (AIP) . After recurrence as AIP with IgG4-SC, CST was resumed. Serum IgG4 level was deteriorated at the maintenance corticosteroid dose at 7.5mg then, enhanced computed tomography indicated thickening of hilar bile duct. Endoscopic retrograde cholangiographic image showed a long stricture of the bile duct, except for the lower bile duct. IDUS showed uniformity thickening of the bile duct wall. Endoscopic examination of the bile duct failed to obtain histopathological diagnosis. Steroid trial was started. At 14 days after CST, ERC showed improvement. Therefore the case was diagnosed as probable IgG4-SC. It is not easy to discriminate IgG4-SC without AIP from primary sclerosing cholangitis (PSC) , and cholangiocarcinoma. This case suggests the effectiveness of IDUS and steroid trial for diagnosis of IgG4-SC.

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