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