1. Overview Diagnosis & Treatment
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 Neither ANA pathogenic nor SMA disease specific
Anti-LKM1
Neither
pathogenic nor
disease specific
Gamma globulin
Autoantibody

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

9. AIH Biochemical Levels don’t reflect treatment ANA response 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. Diagnosis Presence Biochemical Histological Exclusion Wilson disease
HCV
Drugs

14. Diagnostic criteria
Laboratory features

15. Diagnostic criteria
Auto antibodies
Laboratory features

16. Histological findings
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 criteria Difference between definite & probable AIH
Degree of IGg elevation
ANA, SMA, anti-LKM1 levels
Exposures to drugs, alcohol & infections
Presence of evolving reperatoire auto antibodies support probable diagnosis

21. Diagnostic scoring system

22. Diagnostic scoring system

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

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

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

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

27. Liver
Immunity

28. Liver
Drugs
Immunity

29. Treatment
prednisone
Prednisone +
azathioprine

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

31. 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

32. Who should be treated?

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

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

35. 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

36. Regimens : prednisone
End point

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

38. Regimens prednisone+azathioprine
End point

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

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

41. Treatment End Points

42. Pattern of response

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

44. Treatment failure (9%)

45. Incomplete response (13%)

46. 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

47. Relapse
Depends on histology at end point

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

49. 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%)

50. Recommendation
High dose prednisone alone or in combination with azathioprine should be used in treatment failure
Corticosteroids should be considered in the decompensated patients
Liver transplantation should be considered in the decompensated patients unsalvaged by drug therapy

51. thanks