Primary Sclerosing Cholangitis (PSC) (cont.)

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What is the treatment for primary sclerosing cholangitis?

The treatment of PSC is supportive, monitoring the progression of the disease and treating symptoms and complications as they arise. The only "cure" is liver transplantation, which may be an option when the disease progresses to cirrhosis and the liver function is affected.

When some of the larger bile ducts become blocked, there is potential to open them with ERCP (endoscopic retrograde cholangiopancreatography) and balloon dilatation and/or stent placement. A gastroenterologist passes a thin fiberoptic scope with a camera into the mouth and threads it through the stomach into the bile ducts. Should a narrow duct be found, a balloon can be used to dilate the narrowing or a stent can be placed to keep the duct open. This is similar to how a cardiologist opens a blocked blood vessel in the heart

In some cases, surgery may be an option to remove some scarred and blocked bile ducts and having more normal bile ducts reconnected bypassing scarred areas of the liver.

Medications

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There is no medication that is approved to treat primary sclerosing cholangitis, but medications may be used to control symptoms. Ursodiol (Actigal), also known as ursodeoxycholic acid (UDCA), may improve liver function tests but has not been shown to increase survival and may be associated with complications like bleeding. While it is thought that PSC may be an autoimmune disease, immunosuppressive medications have not been shown to work.

Itching is often treated with antihistamines including diphenhydramine (Benadryl), hydroxyzine (Atarax) and cyproheptadine (Periactin). Cholestyramine (Questran, Questran Light), a medication that helps bind bile salts may also be helpful.

Should infection occur, treatment may require antibiotics.

As the disease progresses, the damaged liver may not be able to help with the absorption of vitamins and nutrients from the intestine. Vitamins and other dietary and calorie supplements may be required to treat malnutrition.

Medically Reviewed by a Doctor on 4/23/2014

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