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Autoimmune Hepatitis

Autoimmune Hepatitis. Overview Diagnosis & Treatment. Liver. Immunity. Genetic factors. Triggering factors. AIH. Immuno- regulatory. Autoantigens. Autoimmune Hepatitis (AIH). Unresolving inflammation of the liver characterized by a loss of tolerance against hepatic tissue. AIH.

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Autoimmune Hepatitis

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  1. Autoimmune Hepatitis Overview Diagnosis & Treatment

  2. Liver Immunity

  3. Genetic factors Triggering factors AIH Immuno- regulatory Autoantigens

  4. Autoimmune Hepatitis(AIH) • Unresolving inflammation of the liver characterized by a loss of tolerance against hepatic tissue.

  5. AIH Biochemical Histological Gamma globulin Autoantibody Interface hepatitis Portal plasma cell

  6. AIH Biochemical ANA SMA Anti-LKM1 Gamma globulin Autoantibody

  7. AIH Biochemical ANA SMA Anti-LKM1 Neither pathogenic nor disease specific Gamma globulin Autoantibody

  8. AIH Biochemical ANA SMA Anti-LKM1 Expression Vary during AIH course Don't predict histologic injury Gamma globulin Autoantibody

  9. AIH Biochemical ANA SMA Anti-LKM1 Levels don’t reflect treatment response Do not need monitoring Gamma globulin Autoantibody

  10. AIH Conventional Ab Evolving Ab Biochemical ANA SMA Anti-LKM1 Anti-AGRA Anti-LC1 Anti-SLA/LP pANCA Anti-Actin Gammaglobulin Autoantibody

  11. AIH Histological Neither is disease specific Absence do not preclude diagnosis Interface hepatitis Portal plasma cell

  12. Liver biopsy? • Establish diagnosis • Disease severity • Need for treatment • Therapeutic monitoring

  13. Presence Biochemical Histological Exclusion Wilson disease HCV Drugs Diagnosis

  14. Diagnostic criteria Laboratory features

  15. Diagnostic criteria Auto antibodies Laboratory features

  16. Diagnostic criteria Histological findings Auto antibodies Laboratory features

  17. Diagnostic criteria No toxic or alcohol injury Histological findings Auto antibodies Laboratory features

  18. Diagnostic criteria No active viral infection No toxic or alcohol injury Histological findings Auto antibodies Laboratory features

  19. Diagnostic criteria No genetic liver disease No active viral infection No toxic or alcohol injury Histological findings Auto antibodies Laboratory features

  20. Diagnostic scoring system

  21. Diagnostic scoring system

  22. Definite Pre Rx : >15 Post Rx: >17 Probable Pre Rx : 10-15 Post Rx: 12-17 Diagnostic scoring system

  23. Recommendations • Aminotransferase,gamma globulin levels • ANA &/or SMA – anti LKM1 • Liver tissue exam

  24. Recommendations • AIH diagnostic criteria applied to all patients • Scoring method if AIH diagnosis is not clear

  25. Treatment • Improves • Symptoms • Laboratory tests • Histological findings • Survival (20y life expectancy>80%)

  26. Liver Immunity

  27. Liver Drugs Immunity

  28. Treatment prednisone Prednisone + azathioprine

  29. Treatment prednisone Prednisone + azathioprine *cyclosporine *ursodeoxycholic acid *FK506 *6 mercaptopurine *methotrexate *cyclophosphamide *mycophenolate mofetil *rapamycin

  30. Who should be treated? • Severe disease progress to cirrhosis in 82% within 5 years & mortality is 45% • Mild/moderate disease progress to cirrhosis in 49% within 15 years & a 10 years survival of 90% • Untreated patients with interface hepatitis have 17% probability of cirrhosis within 5 years and normal 5 years life expectancy

  31. Who should be treated?

  32. Mild disease • AST/G globulin • <absolute criteria • Interface hepatitis

  33. Mild disease • AST/G globulin • <absolute criteria • Interface hepatitis Benefit-risk ratio undefined Clinical judgment

  34. Recommendation • Severe disease • Symptomatic disease • Interface hepatitis alone does not compel treatment • Treatment not indicated in patients with inactive cirrhosis, preexistent comorbid conditions • Treatment in most children

  35. Regimens : prednisone End point

  36. Regimens : prednisone End point Cytopenia Thiopurine methyl transferase deficiency Malignancy

  37. Regimens prednisone+azathioprine End point

  38. Regimens prednisone+azathioprine End point Postmenopause Osteoporosis Brittle DM Obesity Hypertension Emotional lability

  39. There is no prescribed minimum or maximum duration of treatment • Therapy should not be instituted with the intention of being indefinite

  40. Treatment End Points

  41. Pattern of response

  42. Pattern of response • No symptoms • Normal billirubin/glob • AST<2UN • Normal tissue • No interface hepatitis

  43. Treatment failure(9%)

  44. Incomplete response(13%)

  45. Relapse Occurs in 20-100% Depends on histology at end point Liver biopsy prior to termination is preferred but not essential Increase AST>3folds Increase gamma globulin>2g/dl

  46. Relapse • Depends on histology at end point

  47. Management after relapse • Indefinite low dose prednisone • Indefinite azathioprine *87% remission *12% were able to be withdrawn from medication(6y)

  48. Liver transplantation • Indicated if deterioration occurs during or after corticosteroid treatment (10%) • 5 year patient & graft survival 83-92% • Auto antibodies disappear within 1y • Disease recurrence is mild (10-35%)

  49. thanks

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