Skip to main content

Health Encyclopedia

Search the Health Encyclopedia

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 people 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 (gallstones in the bile duct)
  • Infections in the liver, gallbladder, and bile ducts

Symptoms

The first symptoms are usually:

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

However, some people have no symptoms.

Other symptoms may include:

Exams and Tests

Even though some people do not have symptoms, blood tests shows that they have abnormal liver function. Your health care provider 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 liver enzymes (liver function tests).

Treatment

Medicines that may be used include:

  • Cholestyramine (such as Prevalite) to treat itching Ursodeoxycholic acid (ursodiol) to improve liver function
  • Fat-soluble vitamins (D, E, A, K) to replace what is lost from the disease itself
  • Antibiotics to treat infections in the bile ducts
  • Medicines to quiet the immune system (such as prednisone, azathioprine, cyclosporine, or methotrexate)

These surgical procedures may be done:

  • 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 bile ducts
  • Proctocolectomy (removal of colon and rectum, for those who have both ulcerative colitis and sclerosing cholangitis)
  • Liver transplant

Outlook (Prognosis)

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

  • Ascites (buildup of fluid in the space between the lining of the abdomen and abdominal organs) and varices (enlarged veins)
  • Biliary cirrhosis (inflammation of the bile ducts)
  • Liver failure
  • Persistent jaundice

Some people 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. People who also have inflammatory bowel disease may have an increased risk of developing cancer of the colon or rectum and should have periodic colonoscopy.

Possible Complications

Complications may include:

References

Gordon FD. Primary sclerosing cholangitis. Surg Clin North Am. 2008;88:1385-407.

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: Elsevier Saunders; 2010:chap 68.

Sabbaghian S, Ahrendt SA. Primary Sclerosing Cholangitis. In: Cameron JL, Cameron AM, eds. Current Surgical Therapy. 11th ed. Philadelphia, PA: Elsevier Saunders; 2014:409-13.

Wang C, Pressman A. Primary Sclerosing Cholangitis. In: Ferri FF, ed. Ferri's Clinical Advisor 2015. 1st ed. Philadelphia, PA: Elsevier Mosby; 2015:section I.

Review Date:4/20/2015
Reviewed By:Subodh K. Lal, MD, Gastroenterologist with Gastrointestinal Specialists of Georgia, Austell, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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

The Agency for Health Care Administration (Agency) and this website do not claim the information on, or referred to by, this site is error free. This site may include links to websites of other government agencies or private groups. Our Agency and this website do not control such sites and are not responsible for their content. Reference to or links to any other group, product, service, or information does not mean our Agency or this website approves of that group, product, service, or information.

Additionally, while health information provided through this website may be a valuable resource for the public, it is not designed to offer medical advice. Talk with your doctor about medical care questions you may have.

Health
Outcome Data

No data available for this condition/procedure.

Read More

Biliary system

Cirrhosis *

Crohn disease


* Has Related Health Outcome Information

Health Encyclopedia

More Features

We Appreciate Your Feedback!
1. Did you find this information useful?
         Yes
         No
2. Would you recommend this website to family and friends?
         Yes
         No