The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan.
The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan.
J Hepatol. 2014 Sep;61(3):690-5. doi: 10.1016/j.jhep.2014.04.016. Epub 2014 Apr 24.
IgG4 related cholangiopathy, a distinctive type of cholangitis of unknown origin, is characterized by increased serum levels of IgG4, massive infiltration of IgG4-positive plasma cells with storiform fibrosis and/or obliterative phlebitis in the thickened bile duct wall, and good response to steroids. Patients with IgG4-cholangiopathy are frequently associated with autoimmune pancreatitis; IgG4-cholangiopathy is recognized as a biliary manifestation of IgG4-related disease. This condition can be diagnosed by a combination of imaging, serology, histopathology, and steroid responsiveness; however, cholangiographic features are often difficult to differentiate from primary sclerosing cholangitis, pancreatic cancer, or cholangiocarcinoma. The Japanese clinical diagnostic criteria for IgG4-related sclerosing cholangitis established in 2012 are useful in the diagnosis of IgG4-cholangiopathy. Although the precise pathogenic mechanism remains unclear, the development of IgG4-cholangiopathy may involve: susceptible genetic factors, abnormal innate and acquired immunity, decreased naïve regulatory T cells, and specific B cell responses. Further studies on genetic backgrounds, disease specific antigens, and the role of IgG4 are necessary to clarify the pathogenesis.
IgG4 相关胆管病是一种独特类型的不明原因胆管炎，其特征为血清 IgG4 水平升高、大量 IgG4 阳性浆细胞浸润伴有纤维分隔样增生和/或增厚胆管壁中的闭塞性静脉炎，且对类固醇治疗反应良好。IgG4 相关胆管病患者常伴有自身免疫性胰腺炎；IgG4 相关胆管病被认为是 IgG4 相关疾病的胆道表现。这种疾病可以通过影像学、血清学、组织病理学和类固醇反应性相结合来诊断；然而，胆管造影特征常常难以与原发性硬化性胆管炎、胰腺癌或胆管癌相区分。2012 年制定的日本 IgG4 相关硬化性胆管炎临床诊断标准有助于 IgG4 相关胆管病的诊断。尽管确切的发病机制仍不清楚，但 IgG4 相关胆管病的发生可能涉及：易感遗传因素、先天和获得性免疫异常、幼稚调节性 T 细胞减少和特异性 B 细胞反应。需要进一步研究遗传背景、疾病特异性抗原和 IgG4 的作用，以阐明发病机制。
