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Long-term treatment reduction and steroids withdrawal in children with autoimmune hepatitis: a single centre
Jérôme Dumortier1, Carlos Torres Arita, Christine Rivet
1Fédération des Spécialités Digestives bUnité d'Hépatologie, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France. jerome.dumortier@chu-lyon.fr
Insights
Long-term corticosteroid withdrawal is achievable in most children with autoimmune hepatitis (AIH). This study shows successful dose reduction and discontinuation of steroids in a large pediatric cohort, offering hope for improved treatment outcomes.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Immunology
Background:
- Autoimmune hepatitis (AIH) in children is typically managed with corticosteroids and azathioprine.
- Long-term corticosteroid use in pediatric AIH presents challenges regarding dose reduction and withdrawal.
- This study evaluates the feasibility of reducing and withdrawing corticosteroids in a large cohort of children with AIH.
Purpose of the Study:
- To describe the experience of long-term corticosteroid dose reduction and withdrawal in pediatric autoimmune hepatitis.
- To assess the rate of successful corticosteroid withdrawal in children with AIH.
- To evaluate treatment outcomes and maintenance therapy in this cohort.
Main Methods:
- Retrospective analysis of pediatric patients diagnosed with AIH between 1990 and 2006.
- Inclusion of all children presenting with AIH at the institution.
- Review of treatment regimens, including corticosteroid and azathioprine use, and patient outcomes.
Main Results:
- The cohort comprised 55 children with type 1, type 2, or seronegative AIH.
- Complete remission was achieved in 69% of patients within 1 year.
- Progressive corticosteroid dose reduction was implemented, with withdrawal possible in 90% of patients by 10 years; azathioprine was maintained in 80%.
Conclusions:
- Long-term corticosteroid withdrawal is feasible in a significant majority of children diagnosed with autoimmune hepatitis.
- These findings support the potential for optimizing treatment strategies to minimize steroid exposure in pediatric AIH.
- Successful management involves careful dose titration and consideration of maintenance immunosuppression.
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.
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