协和医学杂志

2019, v.10(03) 201-205

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IgG4相关性硬化性胆管炎:肝胆胰外科医生应该重视的内科疾病
IgG4-related Sclerosing Cholangitis: An Internal Disease Calls for the Attention of Hepatic-biliary-pancreatic Surgeons

刘乔飞;刘卫;洪涛;张宁;曲强;何小东;
LIU Qiao-fei;LIU Wei;HONG Tao;ZHANG Ning;QU Qiang;HE Xiao-dong;Department of General Surgery,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College;

摘要(Abstract):

IgG4相关性疾病(IgG4-related disease,IRD)是一类近10余年逐渐被认识的以血清IgG4升高、席纹状纤维化以及大量IgG4阳性浆细胞浸润为特征的多器官和系统受累的自身免疫性疾病。胆道是IRD常受累的器官之一。IgG4相关性硬化性胆管炎(IgG4 related sclerosing cholangitis,IRSC)是IRD在胆道的临床表现形式。IRSC多有胆管壁局限性增厚、胆管扩张以及梗阻性黄疸等与胆胰恶性肿瘤相类似的临床表现。IRSC对激素敏感,预后良好,合并恶性肿瘤罕见,绝大多数无需手术治疗,但近几年笔者临床工作中仍不时见到因"胆管扩张、梗阻性黄疸"行手术治疗,术后病理证实为IRSC的病例,回顾这些病例的诊治过程,鲜有术前考虑IRSC的情况。临床上也可见到术前因血清IgG4升高,起初误诊为IRSC,错过最佳手术时机,而术后病理证实为胆管癌的病例。故加强对IRSC的宣讲,特别是加深肝胆胰外科医生对IRSC的认识具有重要临床意义。
IgG4-related disease( IRD),a multi-organ involved autoimmune disease,has been newly defined for only about 10 years. It is characterized by elevated serum IgG4,storiform fibrosis,and massive infiltration of IgG4 positive plasma cells in involved organs. Biliary tract is one of the most commonly involved organs. IgG4-related sclerosing cholangitis( IRSC) is the clinical entity of IRD in biliary tract. It always has clinical symptoms similar to biliary-pancreatic malignancy,such as localized biliary wall thickness,dilation of the bile duct,and obstructive jaundice. IRD is sensitive to the treatment of steroids with a satisfactory prognosis and does not warrant surgical treatment. However,in recent years,we met several cases that underwent an operation due to the bile duct stricture and obstructive jaundice,yet with a final pathological report of IRSC. After reviewing these cases,we found that IRSC was seldom considered before surgery. Moreover,there were some cases of biliary malignancy with elevated serum IgG4 that were misdiagnosed to be IRSC,which led to missing the best operative time. Therefore,it is of great clinical importance to widely broadcast the knowledge of IRSC and especially to deepen the understanding of IRSC for the hepatic-biliary-pancreatic surgeons.

关键词(KeyWords): IgG4相关性疾病;IgG4相关性硬化性胆管炎;胆胰恶性肿瘤;梗阻性黄疸
IgG4-related disease;IgG4-related sclerosing cholangitis;biliary-pancreatic malignancy;obstructive jaundice

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作者(Author): 刘乔飞;刘卫;洪涛;张宁;曲强;何小东;
LIU Qiao-fei;LIU Wei;HONG Tao;ZHANG Ning;QU Qiang;HE Xiao-dong;Department of General Surgery,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College;

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