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

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.

Authors

Institutions

Publication Details

Journal
Arthritis & Rheumatology
Published
2026-04-10
DOI
https://doi.org/10.1002/art.70172
Primary Topic
Vasculitis and related conditions
Type
article
Field-Weighted Citation Impact
0.00

Funders

Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

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

Archana Khan, Paul Dancey, Kelly L. Brown, Jonathan Park et al.
Arthritis & Rheumatology
Vasculitis and related conditions
article

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

Archana Khan, Paul Dancey, Kelly L. Brown, Jonathan Park, David A. Cabral, Else S. Bosman, Phil Riley, Raashid Luqmani, Vidya Sivaraman, Susan Shenoi, Alvaro Orjuela, M. Twilt, K. Toor, Samundeeswari Deepak, Linda Wagner‐Weiner, S. C. Li, Sarah Campillo, Simranpreet Kaur Mann, Kimberly Morishita, Tzielan Lee, Katie Harrison, Sirirat Charuvanij, Alan M Rosenberg, Kathryn Cook, Flora McErlane, B. Anne Eberhard, Stacey E. Tarvin, Susan Kim, Michael W. Beresford, Adam Huber, Samuel J Gagne, Marietta de Guzman, Tamara Tanner, Nick McPhate, Sodality Sutnga, Rae S.M. Yeung, James Bistolarides, L. Nandini Moorthy, for the ARChiVe Investigators Network within the PedVas Initiative, Marisa S Klein‐Gitelman, Melissa Elder, Neil Martin
article en

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.

Arthritis & Rheumatology
Western University (CA), Memorial University of Newfoundland (CA), Nationwide Children's Hospital (US), Siriraj Hospital (TH), Lucile Packard Children's Hospital (US), Nottingham University Hospitals NHS Trust (GB), University of British Columbia (CA), University of Liverpool (GB), University of Calgary (CA), University of California, San Francisco (US), University of Toronto (CA), Medical College of Wisconsin (US), Montreal Children's Hospital (CA), Izaak Walton Killam Health Centre (CA), Royal Hospital for Children (GB), Breach Candy Hospital (IN), University of Florida Health (US), Hospital for Sick Children (CA), Janeway Children's Health and Rehabilitation Centre (CA), Nuffield Orthopaedic Centre (GB), Johnson University (US), Nova Scotia Health Authority (CA), Riley Hospital for Children (US), University of Saskatchewan (CA), University of Florida (US), University of Oxford (GB), Alder Hey Children's NHS Foundation Trust (GB), Akron Children's Hospital (US), Birmingham Children's Hospital (GB), Seattle Children's Hospital (US), Cohen Children's Medical Center (US), Lurie Children's Hospital (US), Children's Hospital of Pittsburgh (US), UCSF Benioff Children's Hospital (US), Children's Hospital at Montefiore (US), Comer Children's Hospital (US), Great North Children's Hospital (GB), Texas Children's Hospital (US), Hackensack Meridian Health (US), BC Children's Hospital (CA), SRCC Children’s Hospital (IN), Royal Manchester Children's Hospital (GB)
Vasculitis Foundation, Canadian Institutes of Health Research
Partnerships for the goals
Openalex Percentile: Top 29%
Vasculitis and related conditions
DOIOpen Access PDFOpenAlex
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.