95% of patients with PBC show increased serum antimitochondrial antibodies, which are the characteristic autoantibodies in this disease. These stimulate autoreactive T cells to accumulate around bile ducts and hepatocytes.
Studies will also show increased serum alkaline phosphatase (ALP or ALK-P), which is a marker of cholestasis.
In PBC, and other situations where cholestasis occurs, the ability to transport and breakdown cholesterol is impaired. This yields increased serum cholesterol.
This is the only FDA-approved medication to treat PBC, and works by reducing the rate of intestinal cholesterol absorption. It also works to nonsurgically treat gallstones, while limiting symptoms of cholestasis (jaundice, pruritis).
The onset of this disease is insidious. It eventually leads to liver cirrhosis and an increased risk of hepatocellular carcinoma. Liver transplant is the best form of treatment in patients with end-stage liver disease.
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