- inflammatory liver disease of unknown cause characterised by suppressor T cell defects with autoantibodies directed against hepatocyte surface antigens
- 25% present with acute hepatitis and signs of autoimmune disease (fever, malaise, urticarial rash, polyarthritis, pleurisy or glomerulonephritis)
- Type I: occur in associated with other autoimmune disorder. Positive antinuclear antibodies, anti smooth muscle autoantibody (ASMA) and anti-actin antibodies
- Type II: occurs in association with anti liver/kidney mircosomal (LKM) antibodies. Most frequently in girls and young women.
- Type III: occurs in association with anti soluble liver-kidney antigen
- Liver biopsy : necroinflammatory process (mononuclear infiltrate of portal and periportal areas & piecemeal necrosis, fibrosis or cirrhosis)
- Treatment: prednisolone followed by azathioprine, or liver transplantation
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