像膽管癌的單獨IgG4相關性硬化性膽管炎－－病例報告

Isolated IgG4-Related Sclerosing Cholangitis Mimicking Cholangiocarcinoma: A Case Report

一名82歲男性因間歇性上腹痛一個月而住院，腹部電腦斷層及膽道磁振造影檢查顯示肝內膽管擴大，並在肝內膽管分叉處有一個疑似腫瘤，當時高度懷疑Klatskin腫瘤，並進行手術治療。病理報告顯示膽總管具有豐富的IgG4陽性漿細胞，及淋巴結具有反應性淋巴樣增生，同時血清IgG4濃度升高，確定診斷為IgG4相關性硬化性膽管炎。此疾病對類固醇治療反應良好及初始常懷疑膽管惡性腫瘤，所以IeG4相關性硬化性膽管炎應該考慮予以鑑別診斷。

An 82-year-old man was admitted for intermittent upper abdominal pain that had lasted for one month. Abdominal Computed Tomography and Magnetic Resonance Cholangiopancreatography revealed dilated bilateral intrahepatic bile ducts and a mass-like lesion at the bifurcation of intrahepatic ducts. Klatskin tumor was highly suspected and surgical treatment was performed. Pathological report revealed common bile duct with abundant IgG4-positive plasma cells and lymph nodes with reactive lymphoid hyperplasia. Subsequently, serum IgG4 level found to be substantially increased, leading to a diagnosis of IgG4-related sclerosing cholangitis (IgG4-SC). Because malignant tumors are frequently suspected on initial presentation, clinicians should differentiate malignant tumors from IgG4-SC, because IgG4-SC responds well to steroid therapy.