Primary Sclerosing Cholangitis

Treatment

Since there is no cure for PSC, treatment focuses on controlling symptoms and slowing the progression of the disease.

Medications

• Medications such as cholestyramine, diphenhydramine and rifampin help relieve a common symptom, itching.
• Antibiotics may be prescribed for recurrent infections.
• Vitamin supplements may be given to counter vitamin deficiencies, which occur because the disease renders the body unable to absorb fat. Calcium and vitamin D are used to treat accompanying osteopenia and osteoporosis (weakening of bones).

Endoscopic Procedures

In some instances, endoscopic or X-ray procedures may be performed to open blockages in the common bile duct and improve bile flow. They use instruments to stretch narrowed bile ducts and remove stones. Stents may be inserted to keep the ducts open. These procedures often are done at the same time as the diagnostic procedures. Biopsies of bile duct strictures (narrowings) may be done at the same time.

Liver Transplant

If liver disease progresses to cirrhosis, physicians look at symptoms and test results to determine the extent of liver damage. If the liver is no longer functioning correctly, doctors may make a referral for a transplant. Sometimes it is necessary to wait as long as a year or more before a liver becomes available. Mayo Clinic has the largest liver transplant program in the United States. See liver transplant.

Signs and symptoms of end-stage liver disease that may indicate a liver transplant include:

• Accumulation of fluid in the abdomen (ascites)
• Changes in mental function
• Gastrointestinal bleeding
• Jaundice
• Severe weakness
• Loss of muscle mass
• Bile duct cancer (cholangiocarcinoma)

Research

Mayo Clinic has ongoing research trials evaluating the treatment of PCS.