What is Primary sclerosing cholangitis ?

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Primary sclerosing cholangitis is a liver disease in which the bile ducts

(small tubes through which bile flows from the liver to the gall bladder and

intestines) progressively decrease in size due to inflammation and scarring.

As a result, bile that is normally carried out of the liver accumulates

within the liver, which then damages liver cells.

How common is primary sclerosing cholangitis?

Although primary sclerosing cholangitis has been considered a rare disease,

recent studies suggest that it is more common than originally thought. The

disease may occur alone, but frequently exists in association with

inflammatory diseases of the colon, such as chronic ulcerative colitis.

Primary sclerosing cholangitis affects both sexes, but is more common in men.

What causes primary sclerosing cholangitis?

At the present time, the exact cause is unknown. However, there is currently

no evidence that the disease is inherited, although some people may be more

at risk because of inheriting a certain blood type. Viruses known to cause

hepatitis have not been associated with primary sclerosing cholangitis. In

many cases, this disease occurs in association with inflammatory bowel

disease. This suggests that a common cause for both diseases may exist.

What are the symptoms of primary sclerosing cholangitis?

The course of primary sclerosing cholangitis is unpredictable and not well

understood. A person can have the disease for many years before symptoms

develop. They may include:

itching

fatigue

jaundice (yellowing of the skin and eyes)

episodes of fever and chills

If a person who suffers from primary sclerosing cholangitis develops fever

and chills, he/she should go to the hospital immediately.

How is primary sclerosing cholangitis diagnosed?

Since individuals may have no symptoms whatsoever for many years, the disease

is often diagnosed because of abnormal liver blood tests.

How is primary sclerosing cholangitis treated?

Presently, there is no known specific treatment for primary sclerosing

cholangitis. However, symptoms of this disease can be treated with varying

success by antibiotics, vitamins and medications to control itching, improve

bile flow, etc. Sometimes, insertion of a stent with a procedure known as

endoscopy may be effective in alleviating jaundice and other symptoms. When

the disease has progressed to liver failure, liver transplantation may be

considered. Liver transplantation is successful in 80 to 90 per cent of adult

patients..

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