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Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis Causes

The exact cause of primary sclerosing cholangitis is unknown.

Primary Sclerosing Cholangitis Definition

Primary sclerosing cholangitis is a medical condition characterized by inflammation and scarring of the bile ducts located inside and outside the liver, which causes bile duct blockage, bile accumulation in the liver resulting to damaged liver cells and eventually lead to liver failure.

Primary Sclerosing Cholangitis Diagnosis

Physical symptoms and liver function test results showing extremely elevated blood levels of liver enzymes may suggest diagnosis of primary sclerosing cholangitis. Endoscopic Retrograde Cholangiopancreatography (ERCP) can provide image showing narrowed bile ducts; this method is considered as the gold standard for diagnosing primary sclerosing cholangitis. Magnetic resonance cholangiography provides image of the liver and bile ducts.

Primary Sclerosing Cholangitis Symptoms and Signs

Primary sclerosing cholangitis produces symptoms that are associated with itching, fatigue, abdominal pain, and yellowing of the skin and eyes (jaundice). Chills and fever is commonly present when infection has occurred. There are also cases when primary sclerosing cholangitis produces no symptoms and are only discovered due to abnormally elevated alkaline phosphatase levels (a liver enzyme) during a routine physical examination.

Primary Sclerosing Cholangitis Treatment

Treatment includes medications like Rifampin to diminish itching; antibiotics to control infection; Ursodiol given orally to restore and replenish other bile acids in the body to delay the progression of the disease. For patients diagnosed with advance cirrhosis, treatment usually requires liver transplantation. Surgery is also required for patients who have developed dominant strictures in the extrahepatic bile ducts.

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