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.
Jaundice, weight loss, and abdominal pain are sometimes present.
Diagnosis includes a cholangiogram (an imaging study of the biliary tract), blood tests, and biopsy.
Treatment includes steroids or rituximab.Treatment includes steroids or rituximab.
(See also Overview of Gallbladder and Bile Duct Disorders.)
IgG4-related sclerosing cholangitis (IgG4-SC) resembles primary sclerosing cholangitis (PSC) in that it is the result of an abnormally regulated immune system (IgG4-related disease) affecting the liver and 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 men older than 50 to 60 years old. 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)
Biopsy
Doctors suspect people who have both pancreatitis and cholangiopathy (damage to the bile ducts) of having IgG4-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
Steroids, also sometimes referred to as glucocorticoids or corticosteroids
Sometimes rituximab or inebilizumabSometimes rituximab or inebilizumab
Steroids (usually prednisone) are the medications of choice for IgG4-related sclerosing cholangitis.Steroids (usually prednisone) are the medications of choice for IgG4-related sclerosing cholangitis.
Complete remission, which can be achieved with steroids, is the goal of treatment. If steroid therapy fails, rituximab is used. Inebilizumab, a monoclonal antibody that targets cells thought to play a role in the disease's inflammation and fibrosis (scarring), also reduces disease flares when used after steroid treatment.Complete remission, which can be achieved with steroids, is the goal of treatment. If steroid therapy fails, rituximab is used. Inebilizumab, a monoclonal antibody that targets cells thought to play a role in the disease's inflammation and fibrosis (scarring), also reduces disease flares when used after steroid treatment.
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