2016 Volume 30 Issue 4 Pages 787-798
Sclerosing cholangitis have been classified into IgG4 related sclerosing cholangitis, primary sclerosing cholangitis and secondary sclerosing cholangitis. Obliterative fibrosis is the main cause of biliary stenosis in patients with PSC, which creates short strictures. The luminal side of the bile ducts, including the lining of the biliary epithelial cells, is preferentially affected in patients with PSC. In contrast, severe lymphoplasmacytic infiltration into the bile ducts in the long region is the main cause of biliary stenosis in patients with IgG4-SC, which results in long strictures. Fibroinflammatory involvement is observed mainly in the stroma of the bile duct wall in patients with IgG4-SC, whereas the bile duct epithelium is intact. Cholangiogram by ERCP and bile duct wall changes by IDUS reflect these differences. In addition, bile duct biopsy and cytology are essential to localized strictures in order to rule out bile duct cancer.
