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 1.6 mg/kg/day, and the dose was progressively reduced to 0.32 mg/kg/day at 1 year, 0.24 mg/kg/day at 3 years, 0.11 mg/kg/day at 5 years and 0.05 mg/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.