Primary sclerosing cholangitis is a rare disease with an estimated prevalence in the United States of 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 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 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.
What are the symptoms of primary sclerosing cholangitis?
Most patients with early primary sclerosing cholangitis have no symptoms, and the presence of primary sclerosing cholangitis is recognized only because of abnormally elevated blood levels of liver enzymes (particularly alkaline phosphatase levels) that often are performed along with a routine physical examination.
Early symptoms of primary sclerosing cholangitis include fatigue and bodily itching (pruritus). As the disease progresses, patients may develop jaundice (yellowing of skin and darkening of urine). Jaundice is due to the accumulation of bilirubin in the body. The bilirubin accumulates because it is not able to be eliminated in the bile due to prolonged obstruction of the bile ducts. The accumulation of bilirubin turns the skin and whites of the eye (sclera) yellow. The reason for the pruritus is not entirely known. It may be due to accumulation of bile salts in the body, also as a result of obstruction of the bile ducts.
As primary sclerosing cholangitis progresses, patients typically develop right upper abdominal pain, fever, fatigue, pruritus, and jaundice. These patients also are at risk of developing primary sclerosing cholangitis complications.
The patients with the autoimmune form of primary sclerosing cholangitis have more rapid and early onset of symptoms of abdominal pain, jaundice and fever than the majority of patients with the more indolent form of primary sclerosing cholangitis.