Treatment:

Medications that may be used include:

• Cholestyramine
• Ursodeoxycholic acid (ursodiol)
• Fat-soluble vitamins (D, E, A, K)
• Antibiotics for infections in the bile ducts
• Medications that quiet the immune system (prednisone, azathioprine, cyclosporine, methotrexate)

Surgical procedures:

• Inserting a long, thin tube with a balloon at the end to open up narrowing (endoscopic balloon dilation of strictures)
• Placement of a drain or tube for major narrowing (strictures) of biliary ducts
• Proctocolectomy (for those who have both ulcerative colitis and sclerosing cholangitis)
• Liver transplant

Expectations (prognosis):

How well patients do varies. The disease tends to get worse over time and most patients develop:

• Biliary cirrhosis
• Liver failure
• Persistent jaundice

Some patients develop infections of the bile ducts that keep returning. Patients may have chronic liver disease that gets worse, with ascites and varices.

People with this condition have an increased risk of developing cancer of the bile ducts (cholangiocarcinoma). They should be checked on a regular basis (usually each year) with liver scans and blood tests.

Complications:

• Bleeding esophageal varices
• Cancer in the bile ducts (cholangiocarcinoma)
• Cirrhosis and liver failure
• Infection of the biliary system (cholangitis)
• Narrowing of the bile ducts (strictures)
• Vitamin deficiencies