• Autoimmune disease resulting in antibodies directed against hepatocyte surface antigens
  • There are two types;
    • Type I
      • Affects children and adults (bimodal distribution)
      • Anti-smooth muscle antibodies in 80%
      • ANA positive in 10%
    • Type II
      • Affects children
      • More commonly progresses to cirrhosis
      • Associated with anti-liver/kidney microsomal type I antibodies
  • Associated with;
    • Pernicious anaemia
    • Autoimmune haemolysis
    • UC
      DM
    • Autoimmune thyroiditis

 

Clinical features

  • Predominantly affects young/middle aged women
  • 25% present with acute hepatitis and features of autoimmune disease – fever, malaise, rash, polyarthritis,
  • The remainder present insidiously
  • Complications are those associated with cirrhosis

Tests

  • Abnormal LFTs – raised AST
  • Hypergammaglobulinaemina (IgG)
  • Positive autoantibodies – ANA, SMA, anti-liver/kidney microsomal antibodies
  • Anaemia, reduced WCC and platelets indicated hypersplenism

Management

  • Prednisolone
  • Azathioprine
  • Liver transplantation

Prognosis

  • Overall 80% over ten years, reduced if there is cirrhosis
 

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