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Bile ducts are important because they allow bile to drain out of the liver. In primary sclerosing cholangitis, the bile ducts inside and outside the liver become inflamed and scarred. The ducts become blocked as scarring increases. This results in cholestasis, or the stoppage of biliary flow. Although bile is produced in the liver, it needs to empty into the intestines, where it helps in the digestion process. Bile trapped in the liver damages liver cells and eventually causes liver failure. Primary sclerosing cholangitis is a progressive disease often leading to biliary cirrhosis within 10 to 15 years.
Primary sclerosing cholangitis occurs in approximately 20–60 out of one million people. It affects both males and females, but is more common in men (2:1 ratio). The disease usually begins between ages 30 and 60, but is also increasingly being recognized in children and infants. Approximately 70% of patients with primary sclerosing cholangitis also have inflammatory bowel disease (IBD), more commonly ulcerative colitis than Crohn's disease.
Although the cause of primary sclerosing cholangitis is unknown, researchers are investigating a possible relationship between primary sclerosing cholangitis and IBD. Other theories under investigation include the possible role of bacteria, viruses, and immune system problems.
Many people with primary sclerosing cholangitis have no symptoms when they are first diagnosed; others have already developed chronic liver disease. Especially in childhood primary sclerosing cholangitis, it is highly variable. The most common nonspecific symptom is fatigue. Other less common symptoms include severe itching, weight loss, jaundice, abdominal pain, and unexplained fever. Primary sclerosing cholangitis often progresses slowly, so a person can have the disease for years before symptoms develop.
Primary sclerosing cholangitis is diagnosed through cholangiography. This involves injecting dye into the bile ducts and taking an X-ray. In people with primary sclerosing cholangitis, the cholangiogram usually shows multiple sites in the biliary tree where the bile ducts narrow. In addition, a blood test called a serum alkaline phosphatase level may be done. This test reflects obstruction of bile flow.
In people without symptoms, primary sclerosing cholangitis is usually first suspected when a physician notices enlargement of the liver during a physical exam. Liver function tests then reveal more abnormalities.
A liver biopsy (tissue sample) may be done to confirm primary sclerosing cholangitis and to help determine the severity and extent of the disease. This can be done on an outpatient basis using a local anesthetic.
At this time, no medication has proven to be a fully successful cure for primary sclerosing cholangitis. Treatment of primary sclerosing cholangitis mainly focuses on managing symptoms and slowing the progression of the disease.
Some medications can help relieve itching. Recurrent infections are treated with antibiotics, and vitamin supplements may be given to replace those that are deficient (because of the body's inability to absorb fat). In some cases, surgery is needed to open blockages in the common bile duct.
If treatment no longer controls primary sclerosing cholangitis and severe liver failure occurs, liver transplantation may be an option. Liver transplantation remains the only effective therapeutic option for patients with end-stage liver disease from primary sclerosing cholangitis. The survival rate for this procedure in people with primary sclerosing cholangitis is typically 75 percent or more, and patients experience a good quality of life after recovery.
Learn about other Liver Disease States.
Children's Hospital's main campus is located in the Lawrenceville neighborhood. Our main hospital address is:
Children’s Hospital of Pittsburgh of UPMC
One Children’s Hospital Way
4401 Penn Ave.
Pittsburgh, PA 15224
In addition to the main hospital, Children's has many convenient locations in other neighborhoods throughout the greater Pittsburgh region.
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