Primary Biliary Cirrhosis Definition

An autoimmune disorder in which chronic and progressive inflammation destroys the intrahepatic bile ducts (bile ducts within the liver), blocking the flow of bile. Primary biliary cirrhosis appears to run in families, suggesting a hereditary component. Early symptoms include fatigue, tenderness or pain in the upper right abdomen, and itching (pruritis). Over time, signs of liver damage, such as jaundice and hepatomegaly (enlarged liver), emerge. Primary biliary cirrhosis is most common in women between the ages of 40 and 60.

The diagnostic path includes

  • Liver function tests, which typically show elevations of the enzymes alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (ALP), and gamma-glutamyl transpeptidase (GGTP).
  • Blood tests to measure the level of immunoglobin (elevated) and detect the presence of antimitochondrial antibodies (positive)
  • Imaging procedures such as abdominal ultrasound, computed tomography (ct) scan, or magnetic resonance imaging (mri)
  • Percutaneous liver biopsy

The progressive destruction of the bile ducts results in cholestasis and cirrhosis, leading ultimately to liver failure. Complications include osteoporosis (arising from the body’s inability to metabolize vitamin D and calcium), portal hypertensionesophageal varices, and primary liver cancer.

Primary Biliary Cirrhosis Treatment

There are currently few medical treatment options. The medication ursodiol (Actigall), sometimes taken to help dissolve gallstones, slows the progression of the inflammatory process in some people. Antihistamine medications can help relieve the itching in the early stages; in later stages some people experience relief from itching with bile sequestrant medications such as cholestyramine and colestipol, which bind with bile in the gastrointestinal tract. The only curative treatment, however, is liver transplantation.


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