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Reliable Isolation of Central Nervous System Microvessels Across Five Vertebrate Groups
Published on: January 12, 2020
Central nervous system vasculitis in pediatric microscopic polyangiitis
Danveer Bhadu1, Puneet Kumar1, Kiran Preet Malhotra2
1King George's Medical University, Lucknow.
Abstract:
Microscopic polyangiitis is a small vessel vasculitis, associated with myeloperoxidaseantineutrophil cytoplasmic antibody. It rarely occurs in children. Central nervous system involvement in pediatric microscopic polyangiitis is not a well known entity with perhaps only five cases till date. We hereby present a 14-year-old girl with arthralgia, seizure, leukocytoclastic vasculitis, interstitial lung disease secondary to recurrent pulmonary hemorrhage, pauci-immune glomerulonephritis and high titers of MPO-ANCA, hence diagnostic of microscopic polyangiitis. Magnetic resonance imaging of brain showed diffuse parenchymal involvement which resolved at six months with development of new foci of microhemorrhages in different stages of evolution, reminiscent of vasculitis.
.Insights
Microscopic polyangiitis is a rare vasculitis in children. This case highlights central nervous system involvement, showing brain lesions that evolved over time.
Area of Science:
- Pediatric Rheumatology
- Neurology
- Nephrology
Background:
- Microscopic polyangiitis (MPA) is a rare small vessel vasculitis.
- It is associated with myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA).
- Central nervous system (CNS) involvement in pediatric MPA is exceptionally rare.
Observation:
- A 14-year-old girl presented with arthralgia and seizures.
- She had leukocytoclastic vasculitis, interstitial lung disease with pulmonary hemorrhage, and pauci-immune glomerulonephritis.
- High MPO-ANCA titers confirmed the diagnosis of MPA.
Findings:
- Brain MRI revealed diffuse parenchymal involvement.
- Lesions resolved within six months.
- New microhemorrhage foci appeared, indicating ongoing vasculitic activity.
Implications:
- This case expands the understanding of CNS manifestations in pediatric MPA.
- It underscores the importance of neuroimaging in diagnosing and monitoring MPA in children.
- The findings suggest a potential for recurrent CNS vascular events in pediatric MPA.

