如何诊断免疫球蛋白G4相关性胆管炎：胆道中的多面手
How to Diagnose Immunoglobulin G4-Associated Cholangitis: The Jack-of-All-Trades in the Biliary Tract.
作者信息
Hubers Lowiek M, Beuers Ulrich
机构信息
Department of Gastroenterology & Hepatology and Tytgat Institute of Liver and Intestinal Research, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
出版信息
Viszeralmedizin. 2015 Jun;31(3):185-8. doi: 10.1159/000431028. Epub 2015 Jun 9.
BACKGROUND
Immunoglobulin (Ig) G4-associated cholangitis (IAC) is an inflammatory disorder of the biliary tract displaying characteristic features of IgG4-related disease (IgG4-RD): elevation of IgG4 serum levels, infiltration of IgG4+ plasma cells in the affected tissue, and good response to immunosuppressive treatment.
METHODS AND RESULTS
The clinical presentation of IAC is often misleading, mimicking other diseases of the biliary tract such as cholangiocarcinoma or primary and secondary sclerosing cholangitis. The HISORt criteria form the cornerstone in the diagnosis of IAC, combining histopathological (H), imaging (I), and serological (S) features including serum IgG4, other organ manifestations (O) of IgG4-RD and response to treatment (Rt). The accuracy of the HISORt criteria is limited. Novel diagnostic approaches are under evaluation.
CONCLUSION
More accurate biomarkers are needed to correctly diagnose IgG4-RD and prevent misdiagnoses and unnecessary therapeutic interventions.
背景
免疫球蛋白（Ig）G4相关性胆管炎（IAC）是一种胆道炎症性疾病，具有IgG4相关疾病（IgG4-RD）的特征：血清IgG4水平升高、受累组织中IgG4+浆细胞浸润以及对免疫抑制治疗反应良好。
方法与结果
IAC的临床表现常具有误导性，可模仿其他胆道疾病，如胆管癌或原发性和继发性硬化性胆管炎。HISORt标准是IAC诊断的基石，它结合了组织病理学（H）、影像学（I）和血清学（S）特征，包括血清IgG4、IgG4-RD的其他器官表现（O）以及对治疗的反应（Rt） 。HISORt标准的准确性有限。新型诊断方法正在评估中。
结论
需要更准确的生物标志物来正确诊断IgG4-RD，并防止误诊和不必要的治疗干预。
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