Intrahepatic biliary dysplasia

Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis (PSC) is a rare inflammatory disease of unknown origin that progressively damages and blocks bile ducts inside and outside the liver. Inflammation of the bile ducts leads to scar formation and narrowing of the ducts over time. As scarring increases, the ducts become blocked. As a result, bile builds up in the liver and damages liver cells. Eventually, scar tissue can spread throughout the liver, causing cirrhosis and liver failure.

The prevalence of PSC in the U.S is estimated to be ≈6.3 per 100,000.* Most people with PSC are adults but the disease also occurs in children. The average age at diagnosis is 40. PSC is more common in men than women. Having family members with PSC may increase a person's risk for developing PSC.

The main symptoms of PSC are itching, fatigue, and jaundice. PSC can lead to various complications, including:

deficiencies of vitamins A, D, E, and K
infections of the bile ducts
cirrhosis
liver failure
bile duct cancer

The first step in diagnosing PSC is usually blood tests to check levels of liver enzymes. Endoscopic retrograde cholangiopancreatography (ERCP) is often the confiratory test. ERCP injects radio-opaque dye into the bile ducts. X rays are taken when the dye is injected. ERCP also can be used to take a tissue sample or to treat blocked ducts. Magnetic resonance cholangiopancreatography (MRCP) may also be used to obtain pictures of the bile ducts. Ultrasound can also be used to visualize the liver to obtain a liver biopsy.

Treatment is supportive with the goal of treating symptoms and complications. Liver transplant may be an option in the event of liver failure.