Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis - a progressive and chronic condition in which segments of the BILE DUCTS become inflamed, causing SCAR tissue (sclerosis) that narrows and stiffens them. The scarring reduces and eventually destroys the ability of the ducts to carry BILE. About 75 percent of people who have primary sclerosing cholangitis also have INFLAMMATORY BOWEL DISEASE (IBD), suggesting a related autoimmune process. Primary sclerosing cholangitis is most common in men between the ages of 20 and 40.

In the early stages of the disease symptoms are mild and tend to wax and wane. Early symptoms may include fatigue, tenderness or PAIN in the upper right abdomen, and mild JAUNDICE (yellowish discoloration of the skin). Often the discovery of primary sclerosing cholangitis comes with elevated enzyme levels on LIVER FUNCTION TESTS done for other reasons, with confirmation by imaging procedures, such as ULTRASOUND or COMPUTED TOMOGRAPHY (CT) SCAN, and PERCUTANEOUS LIVER BIOPSY. As more damage to the bile ducts occurs, symptoms intensify.

Medical interventions for primary sclerosing cholangitis are primarily supportive and aim to relieve symptoms such as PRURITIS (intense itching), which become more prominent as the condition progresses. The damage ultimately results in CIRRHOSIS and LIVER FAILURE. A significant risk during this progression is cholangiocarcinoma, a cancerous tumor that develops in the inflamed bile ducts. The course of the disease varies though tends to run about 10 years from onset of symptoms to liver failure. The only curative treatment is LIVER TRANSPLANTATION.

See also AUTOIMMUNE DISORDERS; BILIARY ATRESIA; CANCER RISK FACTORS; GALLBLADDER DISEASE; PRIMARY BILIARY CIRRHOSIS.

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The Gastrointestinal System

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