IgG4 相关性硬化性胆管炎：一个肝胆胰外科医生应该重视的内科疾病

Subjects: Medicine, Pharmacy >> Preclinical Medicine submitted time 2018-06-14 Cooperative journals: 《协和医学杂志》

刘乔飞 刘卫 洪涛 张宁 曲强 何小东

Abstract： IgG4-related disease (IRD) is a newly defined multi-organ involved autoimmune disease only about 10 years ago. 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 common involved organs.IgG4-related sclerosing cholangitis (IRSC) is the clinical entity of IRD in biliary tract.It always has the similar clinical symptoms to biliary-pancreatic malignancy, such as localized biliary wall thickness, dilation of bile duct and obstructive jaundice. It is sensitive to steroids with satisfactory prognosis, which does not warrant surgical treatment. However, recent years, the authors met several cases who underwent operation due to bile duct stricture and obstructive jaundice. However, the final pathological examinations reported that they were IRSC. After reviewing these medical cases, the authors found IRSC was seldom considered before surgery. As well, there were some other cases of biliary malignancy with elevated serum IgG4 which were misdiagnosed to be IRS, leading to missing the best operative time. Therefore, to widely broadcast the knowledge of IRSC and especially to deepen the understanding of IRSC for the hepatic-biliary-pancreatic surgeons are of great clinical importance.

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