Increased sensitivity of the European Medicines Agency algorithm for classification of childhood granulomatosis with polyangiitis

América G. Uribe, Adam M. Huber, Susan Kim, Kathleen M. O'Neil, Dawn M. Wahezi, Leslie Abramson, Kevin Baszis, Susanne M. Benseler, Suzanne L. Bowyer, Sarah Campillo, Peter Chira, Aimee O. Hersh, Gloria C. Higgins, Anne Eberhard, Kaleo Ede, Lisa F. Imundo, Lawrence Jung, Daniel J. Kingsbury, Marisa Klein-Gitelman, Erica F. LawsonSuzanne C. Li, Daniel J. Lovell, Thomas Mason, Deborah McCurdy, Eyal Muscal, Lorien Nassi, Egla Rabinovich, Andreas Reiff, Margalit Rosenkranz, Kenneth N. Schikler, Nora G. Singer, Steven Spalding, Anne M. Stevens, David A. Cabral

Research output: Contribution to journalArticlepeer-review

25 Scopus citations


Objective. Granulomatosis with polyangiitis (Wegener's; GPA) and other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare in childhood and are sometimes difficult to discriminate. We compared use of adult-derived classification schemes for GPA against validated pediatric criteria in the ARChiVe (A Registry for Childhood Vasculitis e-entry) cohort, a Childhood Arthritis and Rheumatology Research Alliance initiative. Methods. Time-of-diagnosis data for children with physician (MD) diagnosis of AAV and unclassified vasculitis (UCV) from 33 US/Canadian centers were analyzed. The European Medicines Agency (EMA) classification algorithm and European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society (EULAR/PRINTO/ PRES) and American College of Rheumatology (ACR) criteria for GPA were applied to all patients. Sensitivity and specificity were calculated (MD-diagnosis as reference). Results. MD-diagnoses for 155 children were 100 GPA, 25 microscopic polyangiitis (MPA), 6 ANCA-positive pauciimmune glomerulonephritis, 3 Churg-Strauss syndrome, and 21 UCV. Of these, 114 had GPA as defined by EMA, 98 by EULAR/PRINTO/PRES, and 87 by ACR. Fourteen patients were identified as GPA by EULAR/PRINTO/PRES but not by ACR; 3 were identified as GPA by ACR but not EULAR/PRINTO/PRES. Using the EMA algorithm, 135 (87%) children were classifiable. The sensitivity of the EMA algorithm, the EULAR/PRINTO/PRES, and ACR criteria for classifying GPA was 90%, 77%, and 69%, respectively, with specificities of 56%, 62%, and 67%. The relatively poor sensitivity of the 2 criteria related to their inability to discriminate patients with MPA. Conclusion. EULAR/PRINTO/PRES was more sensitive than ACR criteria in classifying pediatric GPA. Neither classification system has criteria for MPA; therefore usefulness in discriminating patients in ARChiVe was limited. Even when using the most sensitive EMA algorithm, many children remained unclassified. The Journal of Rheumatology

Original languageEnglish (US)
Pages (from-to)1687-1697
Number of pages11
JournalJournal of Rheumatology
Issue number8
StatePublished - Aug 2012


  • Classification criteria
  • Granulomatosis
  • Pediatric rheumatology
  • Polyangiitis
  • Vasculitis

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology


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