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 we consume. If the ducts are blocked, bile builds up in the liver and damages liver cells. Eventually, PSC can cause liver failure.

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.

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.

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