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A.D.A.M. Medical Encyclopedia [Internet]. Atlanta (GA): A.D.A.M.; 2013.

A.D.A.M. Medical Encyclopedia.

Sclerosing cholangitis

Primary sclerosing cholangitis; PSC

Last reviewed: July 18, 2013.

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

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:

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:

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

  1. Gordon FD. Primary sclerosing cholangitis. Surg Clin North Am. 2008;88:1385-1407. [PubMed: 18992601]
  2. 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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The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only — they do not constitute endorsementscof those other sites. © 1997–2011 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

Copyright © 2013, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is an independent audit to verify that A.D.A.M. follows rigorous standards of quality and accountability. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial policy, editorial process and privacy policy. A.D.A.M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only — they do not constitute endorsementscof those other sites. © 1997–2011 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

Copyright © 2013, A.D.A.M., Inc.

What works?

  • There is insufficient evidence to support or refute D‐penicillamine for patients with primary sclerosing cholangitisThere is insufficient evidence to support or refute D‐penicillamine for patients with primary sclerosing cholangitis
    D‐penicillamine has been considered for patients with primary sclerosing cholangitis due to its copper reducing and immunomodulatory potential. The only identified randomised clinical trial did not demonstrate any beneficial effect of D‐penicillamine on the course, complications, and survival of patients with primary sclerosing cholangitis. In addition, its use was associated with a number of adverse events. Therefore, we cannot recommend the use of D‐penicillamine outside randomised trials.
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