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Abstract：
A 69-year-old woman suspected to have IgG4-related sclerosing cholangitis causing bile duct stenosis was transferred from another hospital after diarrhea, eosinophilia, and eosinophilic infiltration were detected and prednisolone was prescribed. Additional biliary imaging suggested primary sclerosing cholangitis, but the IgG4 level and inferior bile duct stenosis were alleviated by steroid therapy, suggesting IgG4-related sclerosing cholangitis. Therefore, prednisolone was continued. Bile duct biopsy findings suggesting adenocarcinoma led to a diagnosis of pancreatoduodenectomy. The latter specimen only displayed evidence of primary sclerosing cholangitis, and prednisolone was discontinued. Intractable cholangitis necessitated left hepatectomy, after which serum alkaline phosphatase levels increased and eosinophilic colitis recurred. The reintroduction of prednisolone effectively managed the diarrhea but only temporarily reversed the alkaline phosphatase elevation. When histologic sections from resection specimens were compared, the hepatectomy specimen exhibited greater eosinophil infiltration than the earlier pancreatoduodenectomy specimen, suggesting eosinophilic cholangiopathy superimposed on primary sclerosing cholangitis.
摘要：
一名怀疑患有IgG4相关性硬化性胆管炎导致胆管狭窄的69岁女子因腹泻后从另一家医院转院,嗜酸性粒细胞增多,检测到嗜酸性粒细胞浸润，并处方泼尼松龙。额外的胆道成像提示原发性硬化性胆管炎，但通过类固醇治疗可以缓解IgG4水平和下胆管狭窄，提示IgG4相关性硬化性胆管炎。因此,继续使用泼尼松龙。胆管活检结果提示腺癌导致胰十二指肠切除术的诊断。后者标本仅显示原发性硬化性胆管炎的证据，和泼尼松龙停药。顽固性胆管炎需要左肝切除术，之后，血清碱性磷酸酶水平升高，嗜酸性粒细胞性结肠炎复发。泼尼松龙的重新引入有效地控制了腹泻，但仅暂时逆转了碱性磷酸酶的升高。当比较切除标本的组织学切片时，肝切除标本比早期胰十二指肠切除标本表现出更大的嗜酸性粒细胞浸润，提示原发性硬化性胆管炎叠加了嗜酸性粒细胞性胆管病。
