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Autoimmune Hepatitis PowerPoint Presentation

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Description : This medical presentation titled ""Autoimmune Hepatitis"" is a case presentation on autoimmune hepatitis.

Tags : Autoimmune, Hepatitis, hepatocellular inflammation, periportal hepatitis, Hypergammaglobulinemia, Serum liver-associated autoantibodies, chronic liver diseases, Epidemiology, Liver Histology, Autoimmune Histology, Diagnosis, Treatment, Hepatocellular Carcinoma

Published on : Apr 24, 2021 | Views : 1928 | Downloads : 5

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Slide 1-

Autoimmune Hepatitis
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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
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Laboratory Data
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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
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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
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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
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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
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Clinical Manifestations Symptoms Fatigue 85% Jaundice 77% Abdominal pain 48% Pruritus 36% Anorexia 30% Polymyalgias 30% Diarrhea 28% Fevers 18%
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Clinical Manifestations Physical Findings Hepatomegaly 78% Jaundice 69% Splenomegaly 32% Spider nevi 58% Ascites 20% Encephalopathy 14% Concurrent immune disease 48%
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Clinical Manifestations Laboratory features Elevated AST 100% Hypergammaglobulinemia 92% Inc immunoglobulin G level 91% Hyperbilirubinemia 83% Alk Phos >2x 33%
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Differential Diagnosis Wilson’s disease A1AT deficiency Hemochromatosis Viral hepatitis Drug induced hepatitis
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Liver Histology
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Autoimmune Histology
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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
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Diagnostic Scoring System for Atypical Autoimmune Hepatitis
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Subclassification of AIH Type I Type II Type III
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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
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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
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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
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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
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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
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Treatment
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Indications for Treatment
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Treatment Regimens
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Reasons for Preference Prednisone Cytopenia TPMT deficiency Pregnancy Malignancy Short course <6 mos Combination Postmenopausal state Osteoporosis Brittle diabetes Obesity Acne Emotional Lability Hypertension
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Treatment Endpoints Remission 10-40% of patients Treatment Failure Incomplete Response Drug Toxicity
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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
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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
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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
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Management of Relapse after Drug Withdrawal Relapse at least twice Indefinite low dose prednisone Indefinite low dose azathioprine
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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
Slide 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
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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
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Case Presentation What would do next?