hepatic and biliary disorders
IgG4 Related Sclerosing Cholangitis
Introduction
IgG4-related sclerosing cholangitis (IgG4-SC) is a rare cholangiopathy that causes symptoms similar to those of primary sclerosing cholangitis. IgG4-SC can manifest with cholangitis and pancreatitis. Diagnosis requires an abnormal cholangiogram, elevated serum IgG4 levels, and histologic analysis. Treatment with corticosteroids leads to dramatic and lasting remission. (See also Overview of Biliary Function .) IgG4-related sclerosing cholangitis (IgG4-SC) is a biliary stricturing disease caused by an IgG4-predominant subepithelial lymphoplasmacytic infiltration of intrahepatic and extrahepatic bile ducts. IgG4-SC is clinically distinct from Primary Sclerosing Cholangitis (PSC) (PSC). It is the hepatic manifestation of 