Skip to Content

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

In this condition, the bile ducts inside and outside the liver become narrowed and scarred.

The cause is often unknown.

The disease is often 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:

  • Choledocholithiasis
  • Toxicity from chemicals, copper, bile acids
  • Infections in the liver, gallbladder, and bile ducts

Symptoms

The first symptoms are usually fatigue, yellowing of the skin and eyes (jaundice), and itching. However, some people may have no symptoms.

Other symptoms may include:

Exams and Tests

Some people do not have symptoms, but blood work reveals they have abnormal liver function. The doctor will rule out diseases that cause similar problems. For example, you will be checked for stones in the gallbladder or biliary tract.

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 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.

Possible Complications

References

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

Tung BY, Kowdley KV. Sclerosing cholangitis and recurrent pyogenic cholangitis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger & Fordtran's Gastrointestinal and Liver Disease. 8th ed. Philadelphia, Pa: Saunders Elsevier;2006:chap 65.

Review Date: 7/6/2009
Reviewed By: George F. Longstreth, MD, Department of Gastroenterology, Kaiser Permanente Medical Care Program, San Diego, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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 endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
adam.com

Disclaimer: The information contained in this website, and its associated websites, is provided as a benefit to the local community, and the Internet community in general; it does not constitute medical advice. We try to provide quality information, but we make no claims, promises or guarantees about the accuracy, completeness, or adequacy of the information contained in or linked to this website and its associated sites. As medical advice must be tailored to the specific circumstances of each patient and healthcare is constantly changing, nothing provided herein should be used as a substitute for the advice of a competent physician. Furthermore, in providing this service, Adventist HealthCare does not condone or support all of the content covered in this site. As an Adventist health care organization, Adventist HealthCare acts in accordance with the ethical and religious directives for Adventist health care services.

Adventist HealthCare · 301-315-3030 · 1801 Research BLVD, Suite 400, Rockville, MD 20850

Submit an Online Information Request