Autoimmune Hepatitisdiagnosis & management
Dr E M Said
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.
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
• 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
classification
• 3 main subtypes
• Based on difference in their immunological markers.
• Not endorsed as a valid clinical entity by the international autoimmune hepatitis group
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.
Type 2 AIH
• More common in Europe and south America.
• Associated with anti-LKM
• Described in paediatrics patient but in Europe 20% are adults
Type 3 AIH
• Is the least established form of the disease.
• Associated with anti-SLA/LP
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.
Interface hepatitis and bridging necrosis in severe type 1 autoimmune hepatitis
Plasma cell infiltration of the portal tracts in type 1 autoimmune hepatitis
• 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.
Diagnostic criteria should be applied to all patients
If the diagnosis of AIH is not clear, scoring system should be used
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
Indications for treatment
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
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.
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
•
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
Thank you
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