Long-term treatment reduction and steroids withdrawal in children with autoimmune hepatitis: A single centre experience on 55 children

BACKGROUND: The combination of corticosteroids and azathioprine is the standard therapy for autoimmune hepatitis. The aim of this study was to describe our experience on long-term corticosteroid doses reducing and withdrawal in a large cohort of children with autoimmune hepatitis (AIH). METHODS: All children presenting with AIH in our institution, from 1990 to 2006, were retrospectively included. RESULTS: The study population included 55 children [38 females, 17 males, median age 8 years (ranging from 0.8 to 15)] with type 1 (74.5%), type 2 (20%) or seronegative (5.5%) AIH. The diagnosis was made in 41 of them at the time of acute hepatitis (75%); the other 14 were diagnosed as chronic liver disease (25%). Treatment consisted of corticosteroids and azathioprine in 45 patients or corticosteroids alone in five patients. Complete remission was obtained within 1 year in 31 (69%) patients. The median initial dose of corticosteroids was 1.6mg/kg/day, and the dose was progressively reduced to 0.32mg/kg/day at 1 year, 0.24mg/kg/day at 3 years, 0.11mg/kg/day at 5 years and 0.05mg/kg/day at 10 years. Corticosteroids withdrawal was possible in 0% of patients at 1 year, 75% at 3 years, 78% at 5 years and 90% at 10 years. At the end of follow-up, azathioprine was maintained in 36 patients (80%). Total treatment withdrawal was obtained in four patients. CONCLUSION: Our results strongly confirm that long-term corticosteroids withdrawal is possible in a large majority of children with autoimmune hepatitis.