Azathioprine monotherapy for maintenance of remission in pediatric patients with autoimmune hepatitis

Autoimmune hepatitis (AIH) is a chronic disorder characterized by unresolving liver inflammation and has a fluctuating clinical course. Standard therapy has been corticosteroids given daily, alone or in combination with azathioprine (AZA). The potential for significant side effects from prolonged corticosteroid use remains high. To date, no pediatric literature describes long-term AZA monotherapy after induction of remission with corticosteroids. We conducted a retrospective chart review from 1990 to 2002, which revealed 8 patients with AIH. Three patients were excluded because of other diseases requiring continued or intermittent use of corticosteroids. The time to complete biochemical remission on corticosteroids and AZA was 230 days (range, 74-288 days). Azathioprine was introduced early following the diagnosis (range, 1-35 days). All 5 patients were successfully weaned off corticosteroids after a median of 378 days and were maintained on AZA monotherapy for a median of 37 months (range, 28-82 months). One patient had a disease exacerbation on AZA monotherapy 75 months after the discontinuation of corticosteroids. She responded quickly to a corticosteroid burst and has been on AZA monotherapy for 7 months. One patient relapsed after self-discontinuing all medications. Long-term remission of AM was possible in our case series with the early introduction and maintenance treatment with AZA as monotherapy.