II. Epidemiology
- Primarily in young women with autoimmune disorders
- Prevalence: 11 to 17 in 100,000 persons
III. Associated Conditions
- Hypergammaglobulinemia (IgG levels up to 3x normal)
IV. Labs
- Transaminases Moderately elevated
- Alanine Aminotransferase (ALT) 150-1000
- Aspartate Aminotransferase (AST) 150-1000
- Cholestasis Labs elevated
- Gamma Glutamyl Transferase (GGT) elevated
- Alkaline Phosphatase
- Liver Function Tests abnormal
V. Diagnosis
- Exclude other liver disease
-
Serum Protein Electrophoresis
- IgG levels 1.2 to 3 times normal
- High Negative Predictive Value for Autoimmune Hepatitis
- Specific Labs (False Negative: 10-20%)
- Antinuclear Antibody (ANA)
- Smooth Muscle Antibody (SMA)
- Anti-LKM Microsome type 1 Antibody
VI. Management
- See Prevention of Liver Disease Progression
-
Corticosteroids
- Significantly improve survival
VII. Course
- Severe progressive liver disease is more typical