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Successful treatment of severe juvenile microscopic polyangiitis with rituximab
J Brunner1, M Freund, M Prelog
1Department of Paediatrics, Innsbruck Medical University, Anichstrasse 35, Innsbruck 6020, Austria. juergen.brunner@uki.at
Abstract:
Microscopic polyangiitis (MPA) previously called hypersensitivity angiitis is a systemic necrotizing vasculitis affecting predominantly small vessels. MPA involves multiple organ systems including the lung, the kidneys, the joints, and the skin. MPA mostly affects adults in their fourth and fifth decade of life. MPA and Wegener;s granulomatosis are grouped together as ANCA-associated vasculitis. MPA is associated with high titre of myeloperoxidase antineutrophil cytoplasmic antibodies (MPO)-ANCA. We present a 14-year-old female patient presented with MPA. She was treated with steroids and cyclophosphamide. After the complication of severe lung involvement, rituximab was administered as immune-modulating treatment. The MPA came to remission. This is the first report of a pediatric patient with MPA treated with rituximab. Rituximab might be a potential therapeutic option for relapsing ANCA associated vasculitis in childhood.
Insights
Microscopic polyangiitis (MPA), a rare vasculitis, can affect children. A pediatric case successfully used rituximab, an immune-modulating therapy, for remission, suggesting its potential in childhood ANCA-associated vasculitis.
Area of Science:
- Rheumatology
- Pediatric Rheumatology
- Immunology
Background:
- Microscopic polyangiitis (MPA) is a systemic necrotizing vasculitis primarily affecting small vessels.
- MPA typically affects adults in their fourth and fifth decades, involving organs like lungs, kidneys, joints, and skin.
- MPA is classified as ANCA-associated vasculitis, often linked to high titers of myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA).
Observation:
- This report details a 14-year-old female diagnosed with MPA.
- Initial treatment with steroids and cyclophosphamide was complicated by severe lung involvement.
- Rituximab was administered as an immune-modulating therapy for the severe lung complication.
Findings:
- The pediatric patient with MPA achieved remission following rituximab treatment.
- This case represents the first reported instance of rituximab use in a pediatric patient with MPA.
- The treatment was effective despite severe lung involvement, indicating successful disease control.
Implications:
- Rituximab may represent a potential therapeutic option for pediatric patients experiencing relapsing ANCA-associated vasculitis.
- This finding expands treatment possibilities for rare autoimmune diseases in childhood.
- Further research into rituximab's efficacy and safety in pediatric vasculitis is warranted.
