2019 Volume 33 Issue 1 Pages 22-31
IgG4-related sclerosing cholangitis (IgG4-SC) is recognized as a biliary manifestation of IgG4-related disease. IgG4-SC can be carefully diagnosed based on the clinical diagnostic criteria for IgG4-SC including following four criteria: characteristic biliary imaging findings, elevation of serum IgG4 levels, coexistence of IgG4-related disease, except those of the biliary tract, and characteristic histologic features. Recently clinical practice guidelines for IgG4-SC have been developed. Cholangiographic classification of IgG4-SC is useful for making the differential diagnosis between IgG4-SC and other diseases. When intrapancreatic bile duct stenosis is detected, pancreatic cancer and cholangiocarcinoma (CC) must be ruled out. If stenosis is demonstrated in the hilar region, primary sclerosing cholangitis, cholangiocarcinoma, and follicular cholangitis should be ruled out. Steroid therapy is standard treatment. Oral prednisolone therapy is started at 0.6mg/kg/d for 2 or 4 weeks, and the dose is then gradually tapered. Although the outcome of most IgG4-SC patients has been reported to be good based on the strong responsiveness of IgG4-SC to steroid therapy, further study is needed to elucidate the long-term outcome.
