Corticosteroids are usually given, for example, prednisolone 30-40 mg daily for one month reducing to a maintenance dose of 10 mg daily over the course of the next few months depending upon the clinical response. Treatment may continue for 2-3 years, sometimes for life.
The effectiveness of therapy is checked by performing liver function tests and, if these are deteriorating despite treatment, by taking liver biopsies on a regular basis. Azathioprine may be added if there is no remission. If steroids are ineffective, cyclosporin may be tried.
Fifty percent of patients relapse and should be restarted on steroids.
In late stage disease unresponsive to treatment, liver transplantation should be considered.
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