Autoimmune Hepatitis


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overview on autoimmune hepatitis.

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Autoimmune Hepatitis

  1. 1. Autoimmune Hepatitis diagnosis & management Dr E M Said
  2. 2. definition <ul><li>unresolving inflammation of the liver of unknown cause. </li></ul><ul><li>Reflex a complex interaction between triggering factors,autoantigens,genetic predisposition and immunoregulatory network. </li></ul><ul><li>Characterized by the presence of interface hepatitis & portal plasma cell infiltration in histological examination,hypergammaglobulinaemia and </li></ul><ul><li>auto antibodies. </li></ul>
  3. 4. epidemiology <ul><li>In northern Europeans </li></ul><ul><li>Annual incidence 1.9/100,000 </li></ul><ul><li>Prevalence 16.9/100,000 </li></ul><ul><li>2.6% of liver transplant </li></ul><ul><li>Female affected more than males </li></ul><ul><li>gender ratio 3.6:1 </li></ul>
  4. 5. <ul><li>40% of patient with untreated severe disease die within 6 month of diagnosis. </li></ul><ul><li>of survivors </li></ul><ul><li>40% will develop cirrhosis, </li></ul><ul><li>54% will develop oesophageal varesis </li></ul><ul><li>acute onset AIH is common 40%,and fulminant presentation with encephalopathy within 8 month is possible </li></ul>
  5. 6. classification <ul><li>3 main subtypes </li></ul><ul><li>Based on difference in their immunological markers. </li></ul><ul><li>Not endorsed as a valid clinical entity by the international autoimmune hepatitis group </li></ul>
  6. 7. Type 1 AIH <ul><li>The most common form of the disease worldwide </li></ul><ul><li>Associated with ANA and/or SMA </li></ul><ul><li>Over 70% are female and over 40% younger age group. </li></ul>
  7. 8. Type 2 AIH <ul><li>More common in Europe and south America. </li></ul><ul><li>Associated with anti-LKM </li></ul><ul><li>Described in paediatrics patient but in Europe 20% are adults </li></ul>
  8. 9. Type 3 AIH <ul><li>Is the least established form of the disease. </li></ul><ul><li>Associated with anti-SLA/LP </li></ul>
  9. 10. Diagnostic criteria <ul><li>Diagnosis require presence of characteristics features & exclusion of other condition that resemble AIH </li></ul><ul><li>All patients must be evaluated for hereditary, infectious and drug induced liver injury. </li></ul><ul><li>Interface hepatitis is the histologic hall mark of the syndrome & portal plasma infiltration typifies the disorder, but neither are specific. </li></ul>
  10. 11. Interface hepatitis and bridging necrosis in severe type 1 autoimmune hepatitis
  11. 12. Plasma cell infiltration of the portal tracts in type 1 autoimmune hepatitis
  12. 13. <ul><li>The diagnosis of AIH require determination of aminotransferase and gamma globulins; detection of ANA and/or SMA or in their absence anti-LKM1,and liver tissue examination. </li></ul>
  13. 14. Diagnostic criteria should be applied to all patients
  14. 15. If the diagnosis of AIH is not clear, scoring system should be used
  15. 16. treatment <ul><li>3 RCT showed improve clinical,histological & survival of severe AIH after use of steroids </li></ul><ul><li>No trial has been performed in patients with less severe disease. </li></ul><ul><li>The indication for steroids treatment in patients with mild disease must be individualized and based on clinical judgment </li></ul>
  16. 17. Indications for treatment
  17. 18. Treatment regimens <ul><li>Two regimens comparable with each other and superior to NSAID's </li></ul><ul><li>Prednisolone alone or lower dose of prednisolone in conjunction with azathioprine </li></ul><ul><li>All patients should be monitored for the development of drug side effect </li></ul>
  18. 22. Treatment end point <ul><li>Conventional treatment regimens should be continued until remission,treatment failure,incomplete response or drug toxicity </li></ul><ul><li>Once disease remission is achieved drug withdrawal should be attempted. </li></ul>
  19. 24. relapse <ul><li>Relapse is common after drug withdrawal </li></ul><ul><li>Patients should be monitored by serum aminotransferase ,bilirubin and gamma globulin level </li></ul><ul><li>Two strategies are used in patients who relapse at least twice. </li></ul><ul><li>Indefinite low dose prednisolone therapy </li></ul><ul><li>Indefinite Azathioprine therapy </li></ul>
  20. 26. Treatment failure <ul><li>High dose prednisolone alone or in combination with azathioprine should be used in treatment failure </li></ul><ul><li>Alternative management include administration of cyclosporine,methotrexate, 6-mercaptopurine or urodexycoliuc acid </li></ul><ul><li>Liver transplantation should be considered in the decompansated patient who is unable to undergo of be salvaged by drug therapy </li></ul>
  21. 28. <ul><li>Thank you </li></ul>