Pediatric rheumatology

Evaluating Renal Disease in Pediatric‐Onset Antineutrophil Cytoplasmic Antibody–Associated Vasculitis: Disease Course, Outcomes, and Predictors of Outcome

Arthritis RheumatolPublished Registry-based cohort study

Authors' conclusion

The majority of patients with pediatric AAV achieve inactive renal disease by 12 months; however, almost half have evidence of damage. Renal function at diagnosis is a strong predictor of renal function at 12 months.

Study context

Population
Pediatric-onset AAV (granulomatosis with polyangiitis (GPA), MPA or antineutrophil cytoplasmic antibody (ANCA)-positive pauci-immune glomerulonephritis), ≤18 years at diagnosis, with renal disease by biopsy or dialysis dependence; ARChiVe/PedVas registry68% female
Participants
145 participants
Exposure
Kidney Disease Improving Global Outcomes (KDIGO) renal function category at diagnosis vs normal renal function
  • Patientsn = 145
Outcome
Inactive renal disease (pediatric vasculitis activity score = 0 or 1)
Follow-up
12 months
Methodological detailsambispective, proportional-odds logistic regression
  • Ambispective
  • Proportional-odds logistic regression

Main finding

As reported in the abstract

Patients

83%

Observational data: associations, not effects.

Secondary outcomes

OutcomeOR (95% CI)P value
Non-normal KDIGO category by renal function at diagnosis · 12 monthsModerate to severely reduced renal functionvs Normal renal functionProportional-odds logistic regression model8.62 (2.31–32.1)P = 0.002
Non-normal KDIGO category by renal function at diagnosis · 12 monthsKidney failurevs Normal renal functionProportional-odds logistic regression model26.3 (6.32–109)P < 0.001
OutcomePatients
Permanent renal damage · 12 months42%

Original publication

Evaluating Renal Disease in Pediatric‐Onset Antineutrophil Cytoplasmic Antibody–Associated Vasculitis: Disease Course, Outcomes, and Predictors of Outcome. Arthritis Rheumatol. Published 14 January 2025. doi:10.1002/art.43071

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