Autoimmune hepatitis is an immune-mediated disease targeting hepatocytes. It is more common in middleaged
Caucasian females, although may affect other patient populations and age groups.
The diagnosis is made according to criteria based on the alkaline phosphatase: ALT ratio, IgG, the presence of
autoantibodies, liver histology, response to therapy, and the absence of a viral, alcohol or drug etiology. More recently a
simplified scoring system has been proposed.
In the absence of treatment, the prognosis is very poor with a 60% three year mortality. There are guidelines on the
indications for treatment and some groups of patients may not require treatment. The main element of treatment is
prednisolone which decreases the 3 year mortality to 10%. Prednisolone is tapered down to 5-10 mg per day, as
monotherapy or in combination with azathioprine. Approximately 80% of patients will respond to therapy with
prednisolone with or without azathioprine and this should be given for at least 2 years.
Remission is defined as an asymptomatic patient with serum aminotransferases that are normal or less than two-fold
elevated, a normal level of IgG and inactive liver histology. Relapse occurs in up to 90% of patients following drug
withdrawal. The sensitivity and specificity of liver histology to predict relapse off treatment is not high. Other treatments
that have been proposed include mycophenolate mofetil, budesonide, cyclosporine A, tacrolimus, 6-MP, methotrexate,
ursodeoxycholic acid, rapamycin and rituximab although experience with all these agents is limited.