Imaging: US and CT
IgG4-related disease (IgG4-RD) is characterized by increased serum levels of IgG4, a lymphoplasmacytic infiltrate composed of IgG4-positive plasma cells, storiform fibrosis, obliterative phlebitis, and mild to moderate eosinophilia . IgG4-related sclerosing cholangitis (IgG4-SC) is a biliary lesion associated with IgG4-RD, which is frequently seen in elderly men, characterized by obstructive jaundice owing to a bile duct stricture and responds well to steroid therapy . Recently, the first clinical diagnostic criteria for IgG4-SC were proposed in Japan . As in the diagnostic items of the 2012 IgG4-SC diagnostic criteria, biliary tract imaging of IgG4-SC reveals diffuse or segmental narrowing of the intrahepatic and/or extrahepatic bile duct associated with thickening of the bile duct wall .
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