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Sclerosing cholangitis

Definition

Sclerosing cholangitis refers to swelling (inflammation), scarring, and destruction of the bile ducts inside and outside of the liver.

Alternative Names

Primary sclerosing cholangitis; PSC

Causes

The cause of this condition is usually unknown.

The disease may be seen in patients who have:

Genetic factors may also be responsible. Sclerosing cholangitis occurs more often in men than women. This disorder is rare in children.

Sclerosing cholangitis may also be caused by:

Symptoms

The first symptoms are usually:

  • Fatigue
  • Itching
  • Yellowing of the skin and eyes (jaundice)

However, some people may have no symptoms.

Other symptoms may include:

Exams and Tests

Some people do not have symptoms, but blood work shows that they have abnormal liver function. The doctor will look for:

  • Diseases that cause similar problems
  • Diseases that often occur with this condition (especially inflammatory bowel disease)
  • Gallstones

Tests that show cholangitis include:

Blood tests include:

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

Outlook (Prognosis)

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

Some patients develop infections of the bile ducts that keep returning.

People with this condition have an increased risk of developing cancer of the bile ducts (cholangiocarcinoma). They should be checked regularly with a liver imaging test and blood tests. Patients who also have inflammatory bowel disease may have an increased risk of cancer of the colon or rectum and should have periodic colonoscopy.

Possible Complications

References

Gordon FD. Primary sclerosing cholangitis. Surg Clin North Am. 2008;88:1385-1407.

Ross AS, Kowdley KV. Sclerosing cholangitis and recurrent pyogenic cholangitis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger & Fordtran's Gastrointestinal and Liver Disease. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2010:chap 68.


Review Date: 7/18/2013
Reviewed By: George F. Longstreth, MD, Department of Gastroenterology, Kaiser Permanente Medical Care Program, San Diego, California. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.
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