This document discusses the diagnosis and management of autoimmune hepatitis. It defines autoimmune hepatitis as unresolving liver inflammation of unknown cause that results from a complex interaction of triggers, autoantigens, genetics, and the immune system. The diagnosis requires the presence of interface hepatitis and portal plasma cell infiltration on histological examination, along with hypergammaglobulinemia and autoantibodies. Treatment involves the use of steroids such as prednisolone, either alone or in combination with azathioprine, to improve clinical outcomes and survival in severe cases of autoimmune hepatitis.

1. Autoimmune Hepatitis diagnosis & management Dr E M Said

2. definition unresolving inflammation of the liver of unknown cause. Reflex a complex interaction between triggering factors,autoantigens,genetic predisposition and immunoregulatory network. Characterized by the presence of interface hepatitis & portal plasma cell infiltration in histological examination,hypergammaglobulinaemia and auto antibodies.

4. epidemiology In northern Europeans Annual incidence 1.9/100,000 Prevalence 16.9/100,000 2.6% of liver transplant Female affected more than males gender ratio 3.6:1

5. 40% of patient with untreated severe disease die within 6 month of diagnosis. of survivors 40% will develop cirrhosis, 54% will develop oesophageal varesis acute onset AIH is common 40%,and fulminant presentation with encephalopathy within 8 month is possible

6. classification 3 main subtypes Based on difference in their immunological markers. Not endorsed as a valid clinical entity by the international autoimmune hepatitis group

7. Type 1 AIH The most common form of the disease worldwide Associated with ANA and/or SMA Over 70% are female and over 40% younger age group.

8. Type 2 AIH More common in Europe and south America. Associated with anti-LKM Described in paediatrics patient but in Europe 20% are adults

9. Type 3 AIH Is the least established form of the disease. Associated with anti-SLA/LP

10. Diagnostic criteria Diagnosis require presence of characteristics features & exclusion of other condition that resemble AIH All patients must be evaluated for hereditary, infectious and drug induced liver injury. Interface hepatitis is the histologic hall mark of the syndrome & portal plasma infiltration typifies the disorder, but neither are specific.

11. Interface hepatitis and bridging necrosis in severe type 1 autoimmune hepatitis

12. Plasma cell infiltration of the portal tracts in type 1 autoimmune hepatitis

13. 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.

14. Diagnostic criteria should be applied to all patients

15. If the diagnosis of AIH is not clear, scoring system should be used

16. treatment 3 RCT showed improve clinical,histological & survival of severe AIH after use of steroids No trial has been performed in patients with less severe disease. The indication for steroids treatment in patients with mild disease must be individualized and based on clinical judgment

17. Indications for treatment

18. Treatment regimens Two regimens comparable with each other and superior to NSAID's Prednisolone alone or lower dose of prednisolone in conjunction with azathioprine All patients should be monitored for the development of drug side effect

22. Treatment end point Conventional treatment regimens should be continued until remission,treatment failure,incomplete response or drug toxicity Once disease remission is achieved drug withdrawal should be attempted.

24. relapse Relapse is common after drug withdrawal Patients should be monitored by serum aminotransferase ,bilirubin and gamma globulin level Two strategies are used in patients who relapse at least twice. Indefinite low dose prednisolone therapy Indefinite Azathioprine therapy

26. Treatment failure High dose prednisolone alone or in combination with azathioprine should be used in treatment failure Alternative management include administration of cyclosporine,methotrexate, 6-mercaptopurine or urodexycoliuc acid Liver transplantation should be considered in the decompansated patient who is unable to undergo of be salvaged by drug therapy

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