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Primary Sclerosing Cholangitis

Primary sclerosing cholangitis is a disease in which the bile ducts inside and outside the liver become narrowed due to inflammation and scarring. This causes bile to accumulate in the liver and can result in damage to liver cells. Although the exact cause of Primary Sclerosing Cholangitis is unknown, genetic and immunologic factors appear to play a role. Primary sclerosing cholangitis has been considered a rare disease, but recent studies suggest that it is more common than previously thought. It may occur alone, but approximately 70% of patients have associated inflammatory bowel disease, particularly ulcerative colitis.

Primary sclerosing cholangitis is more common in men than women. Initially, many individuals have no symptoms and the disease is detected because of abnormal laboratory test results, particularly an enzyme test called alkaline phosphatase. It usually begins in the 30s, 40s, and 50s, and is commonly associated with fatigue, itching and jaundice. Episodes of fever and chills from superimposed infection in the bile ducts occasionally occur and can be distressing symptoms. The diagnosis of Primary sclerosing cholangitis is made by cholangiography, an X-ray test involving injection of dye into the bile ducts. This is usually accomplished by an endoscopic procedure called ERCP (endoscopic retrograde cholangiopancreatography) but also may be done radiologically or surgically.

The course of the disease is unpredictable for the patient, but is generally slowly progressive. The patient may have the disease formany years before symptoms develop. Symptoms may persist at a stable level, be intermittent, or progress gradually. Liver failure may occur after 7-15 years of disease or even longer. Approximately 10% of patients who have the disease on a longstanding basis may develop a superimposed tumor of the bile ducts called cholangiocarcinoma.

There is currently no specific treatment for primary sclerosing cholangitis. Research is under way to determine the effectiveness of a number of medications. The various symptoms of primary sclerosing cholangitis often respond effectively to medications that control itching, antibiotics when recurrent infections occur, and vitamins to replace those that are deficient. In some instances, endoscopic, radiologic, or surgical techniques may be employed to open major blockages in the common bile duct and improve bile flow. When progressive liver failure occurs in spite of these measures, liver transplantation may be indicated. It is associated with a survival rate of 75% or more and a good quality of life after recovery.


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