Autoimmune Hepatitis

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    Autoimmune Hepatitis

    • Characterized by serum autoantibodies, hypergammaglobulinemia, and histologic features of "interface hepatitis".
      • Interface Hepatitis is a pattern of liver injury.
    • Incidence: 1.9-17 / 100,000 persons among white europeans. 

     

    Clinical Features

    • Ranges: from asymptomatic mild disease to fulminant hepatic failure.
    • Common Features:
      • Fatigue, Jaudice, Pruritis
      • Nausea, vomiting, skin rash, joint pain ==> less common. 
    • Labs:
      • AST and ALT are from 2-10x ULN.
        • ALP elevated in 20% of cases w/o biliary injury on histology.
      • Bilirubin and Albumin generally normal, unless severe or cirrhosis present. 
      • Serum Anti-nuclear antibodies (ANA) and Anti-smooth muscle antibodies (Anti-SMA) both positive in 60% cases.
        • Serum titres > 1:40 suggest autoimmune hepatitis.
        • p-ANCA may be helpful (if ANA, SMA and Gamma-Globulin low).
      • Gamma-Globulin levels (> 2.0 g/dL [20g/L])
      • Liver biopsy is typically required for diagnosis.
        • Histologic criteria: interface hepatitis with lymphocytes and plasma cells in portal, periportal, or lobular areas.

    Management

    • Absolute indications for treatment:
      • Absolute indications for treatment:

              1.  AST > 10x normal

              2.  AST > 5x normal + gamma-globulin levels >2x normal.

              3.  Bridging or confluent necrosis on liver biopsy

              4.  Symptomatic disease (fever, nausea, vomiting, jaundice)

    • Induction Therapy:
      • Corticosteroids or combined azathioprine + coricosteroids
      • Remission in 80% of patients (combined = less side effects)
    • Maintenance Therapy
      • Same regimen for 12-18mo to increase likelihood of histologic remission.
    • Cessation of therapy before 12mo = high relapse rates. (despite normal liver chemistry)
    • Azathioprine monotherapy (at minimum doses for remission) for long-term suppression
      • (Because only 20% remain in remission after withdrawal of all medical therapy)
    • Minority require: mycophenolate mofetil, cyclosporine, tacrolimus, transplant if ESLD (20-30% recurrence post-tslt).
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