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
- pruritus (itchy skin)
- jaundice (yellowing of the eyes and skin)
- xanthalasmas (focal collections of cholesterol in the skin)
- fluid retention
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.
Symptomatic treatment of pruritus with cholestyramine. Immunosuppressive therapy in advanced cases. Liver transplantation is very successful in improving quality of life and increasing survival.