Primary Biliary Cirrhosis

Primary biliary cirrhosis is a disease of the liver marked by the slow destruction of the bile ducts. When this ducts are damaged bile builds up in the liver and over time damages the tissue. Over time primary biliary cirrhosis can result in liver failure.

Etiology

The cause of the disease us unknown at this time, but research indicates that there is an immunological basis for the disease, making it an autoimmune disorder.

Signs and symptoms

  • fatigue
  • pruritus (itchy skin)
  • jaundice (yellowing of the eyes and skin)
  • xanthalasmas (focal collections of cholesterol in the skin)
  • fluid retention

Diagnosis

Diagnosis of primary biliary cirrhosis can be made using radiological studies, lab tests, and/or doing a liver biopsy.

Liver function tests may show a high serum alkaline phosphatase. Mitochondrial antibodies are seen in 95% of the cases (M2 antibodies are more specific). ANF and SMA antibodies may also be elevated.

Treatment

Symptomatic treatment of pruritus with cholestyramine. Immunosuppressive therapy in advanced cases. Liver transplantation is very successful in improving quality of life and increasing survival.

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