Epidemiology


Etiology


Pathophysiology


Classification


  • Type 1 AIH (80% of cases): characteristic autoantibodies include antinuclear antibodies (ANAs), anti-smooth muscle antibodies (ASMAs) anti-soluble liver antigen antibodies (anti-SLA)
  • Type 2 AIH: characteristic autoantibodies include anti-liver-kidney microsomal-1 antibodies (anti-LKM-1), anti-liver cytosol antibodies-1 (ALC-1)

Clinical features


Pasted image 20231016163207.png

  • Nonspecific symptoms
    • Fatigue
    • Upper abdominal pain
    • Weight loss
  • Signs of acute liver failure (∼ ⅓ of patients)

Diagnostics


Laboratory tests

  • Liver chemistries: ↑↑↑ ALT and ↑↑ AST, ↑ GGT, normal or ↑ ALP, and ↑ bilirubin
  • Serum antibodies
    • ANA, ASMA: combination is highly specific for type 1 AIH
    • Anti-LKM-1: typically positive in type 2 AIH
  • SPEP: hypergammaglobulinemia (↑ IgG)

Liver biopsy

  • Histological findings:
    • Lymphoplasmacytic interface hepatitis: ongoing inflammatory process with lymphocytic infiltration, bridging or multiacinar necrosis, and fibrotic changes
    • Bile duct changes (e.g., cholangitis, ductal injury)

Treatment