Effective Performance of the 2022 American College of Rheumatology/EULAR Classification Criteria for Antineutrophil Cytoplasmic Antibody–Associated Vasculitis in Pediatric Patients: An ARChiVe Study
Issued Date
2026-01-01
Resource Type
ISSN
23265191
eISSN
23265205
Scopus ID
2-s2.0-105040695774
Pubmed ID
41958151
Journal Title
Arthritis and Rheumatology
Rights Holder(s)
SCOPUS
Bibliographic Citation
Arthritis and Rheumatology (2026)
Suggested Citation
Cabral D.A., Bosman E.S., McPhate N., Mann S.K., Toor K.K., Morishita K.A., Luqmani R., Beresford M.W., Bistolarides J., Campillo S., Charuvanij S., Cook K., Dancey P., de Guzman M., Deepak S., Eberhard B., Elder M., Gagne S., Harrison K., Huber A., Khan A., Kim S., Klein-Gitelman M.S., Lee T.C., Li S.C., Martin N., McErlane F., Moorthy L.N., Orjuela A.H., Park J., Riley P., Rosenberg A.M., Shenoi S., Sivaraman V., Sutnga S., Tanner T., Tarvin S.E., Twilt M., Wagner-Weiner L., Yeung R.S.M., Brown K.L. 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 and Rheumatology (2026). doi:10.1002/art.70172 Retrieved from: https://repository.li.mahidol.ac.th/handle/123456789/117180
Title
Effective Performance of the 2022 American College of Rheumatology/EULAR Classification Criteria for Antineutrophil Cytoplasmic Antibody–Associated Vasculitis in Pediatric Patients: An ARChiVe Study
Author(s)
Cabral D.A.
Bosman E.S.
McPhate N.
Mann S.K.
Toor K.K.
Morishita K.A.
Luqmani R.
Beresford M.W.
Bistolarides J.
Campillo S.
Charuvanij S.
Cook K.
Dancey P.
de Guzman M.
Deepak S.
Eberhard B.
Elder M.
Gagne S.
Harrison K.
Huber A.
Khan A.
Kim S.
Klein-Gitelman M.S.
Lee T.C.
Li S.C.
Martin N.
McErlane F.
Moorthy L.N.
Orjuela A.H.
Park J.
Riley P.
Rosenberg A.M.
Shenoi S.
Sivaraman V.
Sutnga S.
Tanner T.
Tarvin S.E.
Twilt M.
Wagner-Weiner L.
Yeung R.S.M.
Brown K.L.
Bosman E.S.
McPhate N.
Mann S.K.
Toor K.K.
Morishita K.A.
Luqmani R.
Beresford M.W.
Bistolarides J.
Campillo S.
Charuvanij S.
Cook K.
Dancey P.
de Guzman M.
Deepak S.
Eberhard B.
Elder M.
Gagne S.
Harrison K.
Huber A.
Khan A.
Kim S.
Klein-Gitelman M.S.
Lee T.C.
Li S.C.
Martin N.
McErlane F.
Moorthy L.N.
Orjuela A.H.
Park J.
Riley P.
Rosenberg A.M.
Shenoi S.
Sivaraman V.
Sutnga S.
Tanner T.
Tarvin S.E.
Twilt M.
Wagner-Weiner L.
Yeung R.S.M.
Brown K.L.
Author's Affiliation
University of Washington
The University of British Columbia
University of Florida
UCSF School of Medicine
Baylor College of Medicine
Western University
University of Liverpool
University of Toronto Faculty of Medicine
University of Oxford Medical Sciences Division
Medical College of Wisconsin
The Hospital for Sick Children
Memorial University of Newfoundland
Cumming School of Medicine
Robert Wood Johnson Medical School
Nationwide Children’s Hospital
Children's Memorial Hospital
Nottingham University Hospitals NHS Trust
UPMC Children’s Hospital of Pittsburgh
Siriraj Hospital
University of Saskatchewan, College of Medicine
BC Children's Hospital
Centre Universitaire de Santé McGill, Hôpital de Montreal Pour Enfants
Birmingham Children's Hospital
Riley Hospital for Children
Lucile Packard Children's Hospital Stanford
IWK Health Centre
Royal Manchester Children's Hospital
The Children’s Hospital at Montefiore
UCSF Benioff Children‘s Hospital
Alder Hey Children's NHS Foundation Trust
Akron Children's Hospital
Cohen Children’s Medical Center
Comer Children's Hospital - Hyde Park
Great North Children's Hospital
Royal Hospital for Children, Glasgow
Breach Candy Hospital
Joseph M. Sanzari Children's Hospital
SRCC Children’s Hospital
Janeway Children's Hospital
The University of British Columbia
University of Florida
UCSF School of Medicine
Baylor College of Medicine
Western University
University of Liverpool
University of Toronto Faculty of Medicine
University of Oxford Medical Sciences Division
Medical College of Wisconsin
The Hospital for Sick Children
Memorial University of Newfoundland
Cumming School of Medicine
Robert Wood Johnson Medical School
Nationwide Children’s Hospital
Children's Memorial Hospital
Nottingham University Hospitals NHS Trust
UPMC Children’s Hospital of Pittsburgh
Siriraj Hospital
University of Saskatchewan, College of Medicine
BC Children's Hospital
Centre Universitaire de Santé McGill, Hôpital de Montreal Pour Enfants
Birmingham Children's Hospital
Riley Hospital for Children
Lucile Packard Children's Hospital Stanford
IWK Health Centre
Royal Manchester Children's Hospital
The Children’s Hospital at Montefiore
UCSF Benioff Children‘s Hospital
Alder Hey Children's NHS Foundation Trust
Akron Children's Hospital
Cohen Children’s Medical Center
Comer Children's Hospital - Hyde Park
Great North Children's Hospital
Royal Hospital for Children, Glasgow
Breach Candy Hospital
Joseph M. Sanzari Children's Hospital
SRCC Children’s Hospital
Janeway Children's Hospital
Corresponding Author(s)
Other Contributor(s)
Abstract
Objective: To assess the 2022 American College of Rheumatology (ACR)/EULAR classification criteria for antineutrophil cytoplasmic antibody–associated vasculitis (AAV) in children with chronic small-to-medium vessel vasculitis. Methods: A cohort of 574 patients, identified by physician's diagnosis (MD-diagnosis) in A Registry of Childhood Vasculitis, was classified by computation of registry data as having granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), or eosinophilic GPA after applying (1) ACR/EULAR AAV criteria and (2) pediatric-adapted European Medicines Agency (Ped-EMA) classification algorithm (incorporating Ankara GPA criteria). Venn diagrams compared the resulting GPA and MPA cohorts with MD-diagnosis. Sensitivity and specificity of criteria for GPA were evaluated against MD-diagnosis. Fisher exact test evaluated differences in the frequencies of individual clinical features in GPA versus MPA. Results: Comparing ACR/EULAR criteria against the Ped-EMA algorithm for classifying AAV, more patients were classified as GPA or MPA (n = 396 vs 360, respectively), fewer had GPA (n = 261 vs 288, respectively), more had MPA (n = 135 vs 72, respectively), and fewer GPA cases coclassified as MPA (12% vs 28%, respectively); there were more differences between GPA and MPA in Pediatric Vasculitis Activity Score–defined clinical features (n = 14 vs 10, respectively). When classifying GPA by ACR/EULAR or Ankara criteria, sensitivity (74.5% vs 72.1%, respectively) was comparable, and specificity for ACR/EULAR criteria (93.9% vs 79.9%, respectively) was improved. 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. Our findings support the preferential use of ACR/EULAR over Ankara criteria for GPA in pediatrics. (Figure presented.).