Pediatric rheumatology

Effective Performance of the 2022 American College of Rheumatology/ EULAR Classification Criteria for Antineutrophil Cytoplasmic Antibody–Associated Vasculitis in Pediatric Patients: An ARChiVe Study

Arthritis RheumatolPublished Diagnostic accuracy study

Authors' conclusion

The 2022 ACR/EULAR classification criteria for AAV perform at least as well as previous pediatric criteria and provide categorical MPA criteria where none existed previously; the criteria for GPA and MPA now specifically differentiate each other, with more differences between them in the frequencies of clinical features.

Study context

Population
Children with chronic small-to-medium vessel vasculitis in A Registry of Childhood Vasculitis (ARChiVe), identified by physician's diagnosis
Participants
574 children
Comparison
2022 American College of Rheumatology (ACR)/EULAR antibody-associated vasculitis (AAV) criteria vs Ped-EMA algorithm (incorporating Ankara granulomatosis with polyangiitis (GPA) criteria), against physician diagnosis
Outcome
Sensitivity and specificity of criteria for GPA against physician diagnosis
Methodological detailsregistry-based cohort, classification criteria validation
  • Registry-based cohort
  • Classification criteria validation

Performance

As reported in the abstract

Classification of GPA vs MD-diagnosis (ACR/EULAR vs Ankara criteria)
Test or modelSensitivitySpecificity
ACR/EULAR74.5%93.9%
Ped-EMA72.1%79.9%

Sensitivity

ACR/EULAR74.5%
Ped-EMA72.1%

Specificity

ACR/EULAR93.9%
Ped-EMA79.9%

Further findings

GPA cases coclassified as MPA

Events

OutcomeACR/EULARPed-EMA
Patients classified as GPA or MPA396360
Patients classified as GPA261288
Patients classified as MPA13572
PVAS-defined clinical features differing between GPA and MPAFisher exact test1410

Original publication

Effective Performance of the 2022 American College of Rheumatology/ EULAR Classification Criteria for Antineutrophil Cytoplasmic Antibody–Associated Vasculitis in Pediatric Patients: An ARChiVe Study. Arthritis Rheumatol. Published 9 April 2026. doi:10.1002/art.70172

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