IgG4-related sclerosing cholangitis (IgG4-SC) causes symptoms similar to those of primary sclerosing cholangitis: inflammation, fibrosis, and narrowing of the bile ducts within and outside the liver, then eventual blockage and destruction of those ducts. Cirrhosis and liver failure may develop.
(See also Overview of Gallbladder and Bile Duct Disorders.)
IgG4-related sclerosing cholangitis (IgG4-SC) resembles but is different from primary sclerosing cholangitis (PSC) in that it is the result of an abnormally regulated immune system (IgG4-related disease) that affects the bile ducts. (IgG4-SC gets its name from the IgG4 antibodies that leak into the bile ducts both within and outside the liver and cause inflammation and fibrosis.)
This rare disorder primarily affects males in their 60s and 70s. Most people with it also have another immune-mediated condition called autoimmune pancreatitis.
Symptoms of IgG4-SC
Symptoms may be similar to those of primary sclerosing cholangitis or cholangiocarcinoma, including jaundice, weight loss, and abdominal pain.
Diagnosis of IgG4-SC
Cholangiogram
Antibody tests (IgG4)
Histology tests
Doctors suspect people who have both pancreatitis and cholangiopathy (damage to the bile ducts) of having IgG-SC. Diagnosis of IgG4-SC requires an abnormal cholangiogram (an imaging study of the biliary tract), elevated IgG4 levels in blood (found in most, but not all people with the disorder), and a characteristic appearance of cells when tissue is examined using a microscope (biopsy).
Treatment of IgG4-SC
Corticosteroids
