Abstract
Sclerosing cholangitis is characterized by chronic inflammatory diseases of the intra-/extrahepatic bile duct showing fibrosis and infiltration of nonspecific inflammatory cells followed by the thickening of the bile duct wall, and luminal stenosis or dilatation. This inflammatory changes extend into the peribiliary connetive tissue. The lining epithelia of the bile ducts, peribiliary glands and blood vessles supplying the bile ducts are also affected. Several diseases present as sclerosing cholangitis. Primary sclerosing cholangitis is a prototype of sclerosing cholangitis, and is followed by biliary cirrhosis. Recently, IgG4 related sclerosing cholangitis which is frequently associated with type I autoimmune pancreatitis and/or other IgG4-related disease, has emerged and been established. This type of sclerosing cholangitis is associated with dense infiltration of lymphoplasmacytes, particularly IgG4+plasma cells, and this type of sclerosing cholangitis responds well to steroid therapy. In addition, ischemic sclerosing cholangitis, particularly biliary stricture after liver transplantation, biliary atresia, hepatolithiasis, infectious cholangitis and other diseases present as sclerosing cholangitis, and they are grouped as secondary or acquired sclerosing cholangitis.
