The document discusses a case presentation of a 54-year-old woman diagnosed with autoimmune hepatitis, detailing her medical history, laboratory findings, and treatment. Autoimmune hepatitis is characterized by hepatocellular inflammation, elevated liver-associated autoantibodies, and the exclusion of other liver diseases, with treatment primarily involving steroids and azathioprine. The document also covers epidemiology, clinical manifestations, diagnostic criteria, and treatment regimens, along with recommendations for management and prognosis.

1. Autoimmune Hepatitis

2. Case Presentation
► 54 yo woman with abnormal liver function test
 9 years ago patient with ele lfts
 No complaints
 PMH: migraine headaches, arthritis, bilateral tubal
ligation, repair of ganglion cyst
 Meds: Prempro, Imitrex
 SH: rare alcohol, no tobacco
 FH: no history of liver disease
 PE: weight 104 lbs, no stigmata of Chronic liver disease

3. Laboratory Data
AST 214
ALT 272
Alk Phos 74
Total Bili 0.6
Total Protein 8.0
Alb 3.0
ANA 1:1280
ASMA 1:80
AMA -
Viral Serologies -
Ferritin 75

4. Other Data
►Sono: 3 mm CBD, normal gallbladder,
increase echogeneity c/w fatty liver
►Liver Biopsy: moderate piecemeal necrosis
with early fibrosis, expanded portal tracts
with plasma cells
►DX: Autoimmune Hepatitis
►RX: Steroids and Imuran

5. Definition
►Self perpetuating hepatocellular
inflammation of unknown cause
►Characterized by the presence of:
 periportal hepatitis
 Hypergammaglobulinemia
 Serum liver-associated autoantibodies
►Exclusion of other chronic liver diseases

6. Epidemiology
►1.9 cases per 100,000 incidence of
Autoimmune Hepatitis in western Europe
►Frequency of AIH among patients with
chronic liver disease is 11%
►Accounts for 5.9% of transplantations in the
US
Boberg K. 1998: Scad J Gastro;33:99-103

7. Background
►40% of patients with untreated severe
disease die within 6 mos of dx
►40% develop cirrhosis
 54% develop esophageal varices
►20% die of hemorrhage
►An acute onset of illness is seen in 40%
patients
►Prednisone and azathioprine are mainstay of
treatment

8. Clinical Manifestations
►Symptoms
 Fatigue 85%
 Jaundice 77%
 Abdominal pain 48%
 Pruritus 36%
 Anorexia 30%
 Polymyalgias 30%
 Diarrhea 28%
 Fevers 18%

9. Clinical Manifestations
►Physical Findings
 Hepatomegaly 78%
 Jaundice 69%
 Splenomegaly 32%
 Spider nevi 58%
 Ascites 20%
 Encephalopathy 14%
 Concurrent immune disease 48%

10. Clinical Manifestations
►Laboratory features
 Elevated AST 100%
 Hypergammaglobulinemia 92%
 Inc immunoglobulin G level 91%
 Hyperbilirubinemia 83%
 Alk Phos >2x 33%

11. Differential Diagnosis
►Wilson’s disease
►A1AT deficiency
►Hemochromatosis
►Viral hepatitis
►Drug induced hepatitis

12. Liver Histology

13. Autoimmune Histology

14. Diagnosis

15. Diagnostic Criteria
►Clinical criteria
 Presence of characteristic clinical features
 Liver histology
 Exclusion of other diseases
►Scoring criteria
 Assess the strength of the diagnosis
 Pretreatment and post-treatment
 Helpful with variant or atypical forms of AIH

16. Diagnostic Scoring System for
Atypical Autoimmune Hepatitis
Category Factor Score Category Factor Score
Gender female +2 Other immune Non-hepatic of
immune nature
+2
AP:AST >3
<1.5
-2
+2
autoabs Anti-
SLA/LP,actin,LC1
+2
glob >2.0
1.5-2.0
1.0-1.5
<1.0
+3
+2
+1
0
histology Interface hepatitis
Plasma cells
Rosettes
None of above
+3
+1
+1
-5
ANA,SMA, LKM1 >1:80
1:80
1:40
<1:40
+3
+2
+1
0
HLA DR3 or DR4 +1
AMA positive -4 Rx response Remission alone
Remission w/relapse
+2
+3
Viral markers Positive
negative
-3
+3
Pretreatment
definite dx
probable dx
>15
10-15
drugs Yes
no
-4
+1
Post-treatment
definite dx
probable dx
>17
12-17
alcohol <25 gm/d
>60 gm/s
+2
-2

17. Subclassification of AIH
►Type I
►Type II
►Type III

18. Type 1 AIH
►Diagnostic autoantibodies: ANA, ASMA
►Age: Bidmodal (10-20 and 45-70)
►% Women: 78
►% Concurrent immune diseases: 41
►Elevated gamma globulin: +++
►Steroid responsiveness: +++
►Progression to cirrhosis (%): 45

19. Type II AIH
►Diagnostic autoantibodies: LKM1
►Age: Pediatric (2-14), rare adults (4%)
►% Women: 89
►% Concurrent autoimmune disease: 34
►Elevated gamma-globulins: +
►Steroids responsive: ++
►% progression to cirrhosis: 82

20. Type III AIH
►Diagnostic autoantibodies: SLA and LP
►Age: adults (30-50)
►% Women: 90
►% Concurrent autoimmune disease: 58
►Elevated gamma-globulin: ++
►Steroid responsive: +++
►% progression to cirrhosis: 75

21. Prognostic Indices
► Laboratory findings at presentation
 AST>10x nl: 50%, 3-year mortality
 AST>5x nl + GGT>2x; 90%, 10-yr mortality
 AST<10x nl + GGT<2x; 49%, cirrhosis at 15 yr; 10%
10-yr mortality
► Histologic findings at presentation
 Periportal hepatitis: 17%,cirrhosis at 5 yr; Nl 5 yr
survival
 Bridging necrosis: 82%, cirrhosis of 5 yr; 45%, 5-yr
mortality
 Cirrhosis: 58%, 5 yr-mortality

22. Recommendations
►Diagnosis of AIH requires aminotransferase
and globulin levels; detection of ANA +/or
SMA, anti-LKM1; and histology
►Diagnostic criteria for AIH should be applied
to all patients
►If the diagnosis is not clear, a scoring
method should be used

23. Treatment

24. Indications for Treatment
Absolute Relative
Serum AST>10x uln Symptoms (fatigue,
arthralgia, jaundice)
Serum AST>5x uln and
globulin >2x nl
Serum AST and globulin
less than absolute
criteria
Bridging necrosis or
multiacinar necrosis
Interface hepatitis

25. Treatment Regimens
Prednisone only Combination
(Pred + AZA)
Week 1 60 mg 30 mg+50 mg
Week 2 40 mg 20 mg+50 mg
Week 3 30 mg 15 mg+50 mg
Week 4 30 mg 15 mg+50 mg
Maintenance
until endpoint
20 mg 10mg+50 mg

26. Reasons for Preference
► Prednisone
 Cytopenia
 TPMT deficiency
 Pregnancy
 Malignancy
 Short course <6 mos
► Combination
 Postmenopausal state
 Osteoporosis
 Brittle diabetes
 Obesity
 Acne
 Emotional Lability
 Hypertension

27. Treatment Endpoints
►Remission
 10-40% of patients
►Treatment Failure
►Incomplete Response
►Drug Toxicity

28. Remission
► Criteria
 Disappearance of
symptoms
 Normal bilirubin +
globulin levels
 Transaminases normal
or less than 2x
 Normal histology or
minimal inflammation
► Action
 Gradual withdrawal of
prednisone
 Discontinuation of
azathioprine
 Regular monitoring for
relapse

29. Treatment Failure
► Criteria
 Worsening clinical, labs
and histology despite
compliance
 Inc transaminasis by
67%
 Development of
jaundice, ascites or
hepatic encephalopathy
► Action
 Pred 60 mg/d or pred
30 mg/d with aza 150
mg/d x 1 mo
 Reduction of the dose
each month of
improvement until
maintenance levels

30. Incomplete Response
► Criteria
 Some or no
improvement in
clinical,labs and
histology during therapy
 Failure to achieve
remission after 3 years
 No worsening of
condition
► Action
 Reduction of dose to
lowest levels possible to
prevent worsening
 Indefinite treatment

31. Management of Relapse after Drug
Withdrawal
►Relapse at least twice
 Indefinite low dose prednisone
 Indefinite low dose azathioprine

32. Management of Suboptimal
Response to Initial Therapy
►Alternative medications
 Cyclosporine, 6MP, ursodeoxycholic acid,
budesonide, methotrexate, cyclophosphamide
and mycophenolate mofetil
►Liver Transplantation
 5 year graft survival 83-92%
 Disease recurrence is mild and easily managed

33. Hepatocellular Carcinoma
►Uncommon in the absence of cirrhosis or
coexisting hepatitis B or C
►If cirrhosis
 RUQ ultrasound
 Alfa fetoprotein every 6-12 months

34. Recommendations
►High dose prednisone alone or prednisone
and aza should be used in treatment failures
►Corticosteroid therapy should be considered
in the decompensated patient
►Liver transplantation should be considered
in the decompensated patient unable to
undergo salvage therapy

35. Case Presentation
AST 19
ALT 12
AP 54
Total protein/albumin 7.3/4.6
Total bilirubin 0.6
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