Primary Sclerosing Cholangitis (cont.)

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How common is primary sclerosing cholangitis?

Primary sclerosing cholangitis is a rare disease with an estimated prevalence in the United States of 1 to 6 per 100,000 people. It is more common in men then in women; approximately 70% of primary sclerosing cholangitis patients are men. The mean age at diagnosis of primary sclerosing cholangitis is around 40 years of age.

There is a strong association between primary sclerosing cholangitis and chronic ulcerative colitis. Primary sclerosing cholangitis can also occur alone or in association with Crohn's disease, a disease of the small and large intestines that is related to ulcerative colitis.

What causes primary sclerosing cholangitis?

The cause of primary sclerosing cholangitis is not known. A small subset (approximately 10%) of primary sclerosing cholangitis called autoimmune hepatitis overlap with primary sclerosing cholangitis syndrome patients has a rapidly progressive form of the disease with early onset of abdominal pain, fever, and itching that responds dramatically to treatment with corticosteroids. Since corticosteroids (such as prednisone) are medications for treating immune diseases such as ulcerative colitis, Crohn's disease, and systemic lupus erythematosus, this small subset of primary sclerosing cholangitis patients are believed to have an immune disorder causing their primary sclerosing cholangitis.

Medically Reviewed by a Doctor on 2/6/2014

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