Abstract
IgG4-related sclerosing cholangitis (IgG4-SC) is a characteristic type of sclerosing cholangitis with the dense infiltration of lymphocytes and IgG4-positive plasma cells with extensive fibrosis in the bile duct wall. A 82-year-old man with elevated liver function tests was admitted to our hospital for further examination of lower bile duct stricture. Stenosis is located only in the lower part of the common bile duct. Although IgG4-SC is often associated with autoimmune pancreatitis (AIP), pancreatic head was not swollen. Serum IgG4 level was also not elevated. Endoscopic ultrasound-guided fine needle aspiration showed only infiltration of lymphocytes without plasma cells. Because it was difficult to rule out bile duct carcinoma or early stage pancreatic cancer, pancreatioduodenectomy was underwent. Histopathological examination showed marked lymphocytic and IgG4-positive plasma cells infiltration, storiform fibrosis and obliterative phlebitis. Although it was not easy to discriminate type1 IgG4-SC without AIP or other IgG4 related diseases from malignant diseases, a steroid trial performed carefully only by specialists is one option in these cases.
