Abstract
Autoimmune pancreatitis (AIP) and its various extrapancreatic lesions are currently considered to represent manifestations of organs involved IgG4-related systemic sclerosing disease. Although most cases of IgG4-related sclerosing cholangitis are associated with AIP, there are some cases with no apparent pancreatic lesions compatible with AIP. Considering the age of onset, associated diseases, cholangiography, serum IgG4 levels, and steroid responsiveness, IgG4-related sclerosing cholangitis differs from primary sclerosing cholangitis. To diagnose IgG4-related sclerosing disease, it is most important to differentiate from bile duct cancer. IgG4-related sclerosing cholangitis should be diagnosed on the basis of combinations of cholangiography, serum IgG4 levels, endoscopic biopsy from the bile duct, and steroid responsiveness.
