II. Epidemiology

  1. Primarily in young women with autoimmune disorders
  2. Prevalence: 11 to 17 in 100,000 persons

III. Associated Conditions

  1. Hypergammaglobulinemia (IgG levels up to 3x normal)

IV. Labs

  1. Transaminases Moderately elevated
    1. Alanine Aminotransferase (ALT) 150-1000
    2. Aspartate Aminotransferase (AST) 150-1000
  2. Cholestasis Labs elevated
    1. Gamma glutamyl transferase (GGT) elevated
    2. Alkaline Phosphatase
  3. Liver Function Tests abnormal
    1. Albumin depressed
    2. ProTime prolonged
    3. Bilirubin elevated

V. Diagnosis

  1. Exclude other liver disease
  2. Serum Protein Electrophoresis
    1. IgG levels 1.2 to 3 times normal
    2. High Negative Predictive Value for Autoimmune Hepatitis
  3. Specific Labs (False Negative: 10-20%)
    1. Antinuclear Antibody (ANA)
    2. Smooth Muscle Antibody (SMA)
    3. Anti-LKM Microsome type 1 Antibody

VI. Management

  1. See Prevention of Liver Disease Progression
  2. Corticosteroids
    1. Significantly improve survival

VII. Course

  1. Severe progressive liver disease is more typical

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