Department of Clinical and Experimental Medicine, University of Catania, Cannizzaro Hospital, Unit of Internal Medicine, Catania, Italy.
Department of Clinical and Experimental Medicine, University of Catania, Cannizzaro Hospital, Unit of Internal Medicine, Catania, Italy.
Am J Med Sci. 2022 Sep;364(3):359-365. doi: 10.1016/j.amjms.2022.04.016. Epub 2022 Apr 22.
IgG4 related sclerosing cholangitis (IgG4-SC) is a chronic cholestatic autoimmune liver disease, characterized by obstruction of the biliary tract due to IgG4-positive plasma cell infiltrations associated with fibrosis, phlebitis and presence of eosinophils. The diagnosis of the disease is supported by elevation in serum IgG4, radiological imaging and histology. In most of the cases IgG4-SC is associated with a wide spectrum of symptoms and manifestations summarized by the definition of IgG4-related disease (IgG4-RD). When the IgG4-SC causes an interruption of the biliary tract, differential diagnosis with cholangiocarcinoma may become challenging, due to the anatomical difficulty to get an appropriate histological pattern of the disease. Here we are describing a case of a male patient admitted to our hospital for jaundice. The stenosis of biliary ducts shown by radiologic imaging suggested Cholangiocarcinoma but, in the patient, IgG4-SC was definitely diagnosed.
IgG4 相关硬化性胆管炎（IgG4-SC）是一种慢性胆汁淤积性自身免疫性肝病，其特征为 IgG4 阳性浆细胞浸润伴纤维化、静脉炎和嗜酸性粒细胞浸润导致胆道阻塞。该病的诊断依据包括血清 IgG4 升高、影像学和组织学。在大多数情况下，IgG4-SC 与 IgG4 相关疾病（IgG4-RD）的广泛症状和表现有关。当 IgG4-SC 导致胆道中断时，由于解剖学上难以获得疾病的适当组织学模式，与胆管癌的鉴别诊断可能具有挑战性。本文描述了一名因黄疸就诊于我院的男性患者。影像学显示胆管狭窄提示胆管癌，但患者被明确诊断为 IgG4-SC。
