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Sclerosing cholangitis

Primary sclerosing cholangitis; PSC

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

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Digestive system
Bile pathway

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Causes

The cause of this condition is unknown in most cases.

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:

Symptoms

The first symptoms are usually:

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:

Tests that show cholangitis include:

Blood tests include liver enzymes (liver function tests).

Treatment

Medicines that may be used include:

These surgical procedures may be done:

Outlook (Prognosis)

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

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:

Related Information

Bile
Crohn disease
Cirrhosis
Biliary system

References

Bowlus C, Assis DN, Goldberg D. Primary and secondary sclerosing cholangitis. In: Sanyal AJ, Boyer TD, Lindor KD, Terrault NA, eds. Zakim and Boyer's Hepatology. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 43.

Ross AS, Kowdley KV. Primary sclerosing cholangitis and recurrent pyogenic cholangitis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 10th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 68.

Zyromski NJ, Pitt HA. The management of primary sclerosing cholangitis. In: Cameron JL, Cameron AM, eds. Current Surgical Therapy. 12th ed. Philadelphia, PA: Elsevier; 2017:453-458.

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Review Date: 4/23/2017  

Reviewed By: Michael M. Phillips, MD, Clinical Professor of Medicine, The George Washington University School of Medicine, Washington, DC. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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