Towards a histological diagnosis of childhood small vessel CNS vasculitis
Authors' conclusion
Distinct histological features were identified in sv-cPACNS biopsies, including moderate to severe angiocentric inflammatory infiltrates in the cortex or white matter, consisting of CD3, CD4, CD8, and CD20 cells, alongside reactive endothelium with specificity of 95%.
Study context
- Population
- Pediatric brain biopsy specimens, single centrebrain biopsy specimens
- Participants
- 31 / 11 / 11
- Comparison
- Brain biopsies of children with sv-cPACNS vs epilepsy and non-vasculitic inflammatory brain disease controls
- sv-cPACNSn = 31
- Epilepsyn = 11reference
- Non-vasculitic inflammatory brain diseasen = 11reference
- Outcome
- Histological features (standardized brain biopsy scoring instrument) associated with sv-cPACNS
Methodological detailssingle-centre, blinded independent scoring by nine neuropathologists, consecutive full-thickness brain biopsies
- Single-centre
- Blinded independent scoring by nine neuropathologists
- Consecutive full-thickness brain biopsies
Performance
As reported in the abstract
Histological features associated with sv-cPACNS
sv-cPACNSOdds ratio (95% CI)P value
Angiocentric inflammation in the cortex
3.231(0.914–11.420)
P = 0.067Angiocentric inflammation in white matter
3.923(1.13–13.6)
P = 0.031Moderate to severe inflammation in the cortex
5.56(1.02–29.47)
P = 0.046Moderate to severe inflammation in white matter
6.76(1.26–36.11)
P = 0.025- Reactive endothelium
- OR 8.93; P = 0.001
- Specificity of combined features
- 95%
Original publication
Towards a histological diagnosis of childhood small vessel CNS vasculitis. Pediatr Rheumatol Online J. Published 28 December 2024. doi:10.
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