Autoimmune Hepatitis. Case Presentation â– 54 yo woman with abnormal liver function test...

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Transcript of Autoimmune Hepatitis. Case Presentation â– 54 yo woman with abnormal liver function test...

  • Slide 1
  • Autoimmune Hepatitis
  • Slide 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
  • Slide 3
  • Laboratory Data AST214 ALT272 Alk Phos 74 Total Bili 0.6 Total Protein 8.0 Alb3.0 ANA1:1280 ASMA1:80 AMA- Viral Serologies - Ferritin75
  • Slide 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
  • Slide 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
  • Slide 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
  • Slide 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
  • Slide 8
  • Clinical Manifestations Symptoms Fatigue 85% Jaundice 77% Abdominal pain 48% Pruritus 36% Anorexia 30% Polymyalgias 30% Diarrhea 28% Fevers 18%
  • Slide 9
  • Clinical Manifestations Physical Findings Hepatomegaly 78% Jaundice 69% Splenomegaly 32% Spider nevi 58% Ascites 20% Encephalopathy 14% Concurrent immune disease 48%
  • Slide 10
  • Clinical Manifestations Laboratory features Elevated AST 100% Hypergammaglobulinemia 92% Inc immunoglobulin G level 91% Hyperbilirubinemia 83% Alk Phos >2x 33%
  • Slide 11
  • Differential Diagnosis Wilsons disease A1AT deficiency Hemochromatosis Viral hepatitis Drug induced hepatitis
  • Slide 12
  • Liver Histology
  • Slide 13
  • Autoimmune Histology
  • Slide 14
  • Diagnosis
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  • 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
  • Slide 16
  • Diagnostic Scoring System for Atypical Autoimmune Hepatitis CategoryFactorScoreCategoryFactorScore Genderfemale+2 Other immune Non-hepatic of immune nature +2 AP:AST>32.01.5-2.01.0-1.51:801:801:401510-15 drugsYesno-4+1Post-treatment definite dx definite dx probable dx probable dx>1712-17 alcohol 60 gm/s +2-2
  • Slide 17
  • Subclassification of AIH Type I Type II Type III
  • Slide 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
  • Slide 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
  • Slide 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
  • Slide 21
  • Prognostic Indices Laboratory findings at presentation AST>10x nl: 50%, 3-year mortality AST>5x nl + GGT>2x; 90%, 10-yr mortality AST
  • Indications for Treatment AbsoluteRelative 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
  • Slide 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
  • Slide 26
  • Reasons for Preference Prednisone Cytopenia TPMT deficiency Pregnancy Malignancy Short course