Division of Gastroenterology and Hepatology, Louis Stokes Cleveland VAMC, Case Medical Center, 10701 East Boulevard, 111E (W), Cleveland, OH 44106, USA.
Clin Liver Dis. 2013 May;17(2):255-68. doi: 10.1016/j.cld.2012.11.007. Epub 2012 Dec 20.
IgG4-associated cholangitis is the hepatobiliary manifestation of a recently characterized inflammatory systemic disease, associated with increased IgG4 serum levels and IgG4-positive lymphoplasmacytic infiltration. Often, patients present with obstructive jaundice, and imaging reveals stenoses of the extrahepatic or intrahepatic bile ducts, often in association with parenchymal pancreatic findings and irregularities of the pancreatic duct. The histologic findings include lymphoplasmacytic infiltrates, on occasion resulting in tumefactive lesions (which can mimic malignancy), obliterative phlebitis, and fibrotic changes. Steroid treatment is the mainstay of management, but relapse is common after discontinuation of therapy or during tapering of steroids and may require further treatment.
IgG4 相关性胆管炎是一种新近被描述的炎症性系统性疾病在肝胆系统的表现，与 IgG4 血清水平升高和 IgG4 阳性浆细胞浸润相关。患者常表现为梗阻性黄疸，影像学检查显示肝外或肝内胆管狭窄，常伴有胰腺实质病变和胰管不规则。组织学表现包括淋巴浆细胞浸润，有时导致肿块样病变（可能类似于恶性肿瘤）、闭塞性静脉炎和纤维化改变。类固醇治疗是主要的治疗方法，但停药后或类固醇减量期间常复发，可能需要进一步治疗。
