Predictive utility of anti-neutrophil cytoplasmic antibody (ANCA) positivity and antigen specificity in the assessment of kidney disease in paediatric-onset small vessel vasculitis
Authors' conclusion
The results of this study suggest important paediatric-specific differences in the predictive value of ANCA compared with adult patients that should be considered when making treatment decisions in this population.
Study context
- Population
- Children and adolescents with small vessel vasculitis, ARChiVe/Paediatric Vasculitis Initiative registry
- Participants
- 406 children
- Exposure
- MPO-ANCA vs PR3-ANCA vs ANCA-negative paediatric-onset small vessel vasculitis
- ANCA-negativen = 41
- MPO-ANCAn = 129
- PR3-ANCAn = 236reference
- Outcome
- Overall and kidney-specific disease activity at diagnosis and outcomes between 1 and 2 years
Methodological detailsregistry-based (ARChiVe/Paediatric Vasculitis Initiative), generalised linear models
- Registry-based (ARChiVe/Paediatric Vasculitis Initiative)
- Generalised linear models
Main finding
As reported in the abstract
onset
MPO-ANCA vs PR3-ANCAOdds ratio (95% CI)P value
Kidney involvement
2.4(1.3–4.7)
P = 0.008Severe kidney dysfunction (KDIGO stages 4-5)
6.04(2.77–13.57)
P < 0.001Observational data: associations, not effects.
Further findings
| Outcome | P value |
|---|---|
| Improvement in kidney function (improved KDIGO category) in similarly severe kidney disease at onset · within 1 yearMPO-ANCA vs PR3-ANCA · MPO-ANCA more likely to improve | P < 0.001 |
Inactive disease, improvement, damage by 1 year
- No significant differences by ANCA positivity or specificity: inactive disease
- ≈68%
- Improvement
- ≥87%
- Damage
- ≈58%
Relapse · between 1 and 2 years after diagnosis
- No significant differences in likelihood of relapse
- ≈11%
Original publication
Predictive utility of ANCA positivity and antigen specificity in the assessment of kidney disease in paediatric-onset small vessel vasculitis. RMD Open. Published 17 June 2024. doi:10.
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