Primary Sclerosing Cholangitis
In primary sclerosing cholangitis (PSC), the bile ducts inside and outside the liver become inflamed and scarred. As the scarring increases, the ducts become blocked. The ducts are important because they carry bile out of the liver. Bile is a liquid that helps break down fat in food. If the ducts are blocked, bile builds up in the liver and damages liver cells. Eventually, PSC can cause liver failure.
Researchers do not know what causes PSC. Among the theories under investigation are the possible role of bacteria, viruses, and immune system problems. PSC appears to be associated with ulcerative colitis, a type of inflammatory bowel disease.
The disease usually begins between ages 30 and 60, but the disease can also arise during childhood. PSC is more common in men than women. PSC progresses slowly, so a personcan have the disease for years before symptoms develop. The main symptoms are itching, fatigue, and jaundice, which causes yellowing of the eyes or skin. An infection in the bile ducts can cause chills and fever.
PSC is diagnosed through cholangiography, which involves injecting dye into the bile ducts and taking an x ray. Cholangiography can be performed as an endoscopic procedure (endoscopic retrograde cholangiopancreatography, ERCP), through radiology or surgery, or with magnetic resonance imaging (MRI) scans. Treatment includes medication to relieve itching, antibiotics to treat infections, and vitamin supplements, as people with PSC are often deficient in vitamins A, D, and K. In some cases, surgery to open major blockages in the common bile duct is also necessary. Liver transplantation may be an option if the liver begins to fail.
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NIH Publication No. 03-4626