Merck Manual

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IgG4-Related Sclerosing Cholangitis

By

Yedidya Saiman

, MD, PhD, Lewis Katz School of Medicine, Temple University

Reviewed/Revised Aug 2023
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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.

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 Overview of Pancreatitis Pancreatitis is inflammation of the pancreas. The pancreas is a leaf-shaped organ about 5 inches (about 13 centimeters) long. It is surrounded by the lower edge of the stomach and the first... read more 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

Corticosteroids (usually prednisone) are the medications of choice for IgG4-related sclerosing cholangitis.

Complete remission, which can be achieved with corticosteroids, is the goal of treatment. If corticosteroid therapy fails, rituximab is used.

Drugs Mentioned In This Article

Generic Name Select Brand Names
Deltasone, Predone, RAYOS, Sterapred, Sterapred DS
RIABNI, Rituxan, RUXIENCE, truxima
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