%0 Journal Article %A América G. Uribe %A Adam M. Huber %A Susan Kim %A Kathleen M. O'Neil %A Dawn M. Wahezi %A Leslie Abramson %A Kevin Baszis %A Susanne M. Benseler %A Suzanne L. Bowyer %A Sarah Campillo %A Peter Chira %A Aimee O. Hersh %A Gloria C. Higgins %A Anne Eberhard %A Kaleo Ede %A Lisa F. Imundo %A Lawrence Jung %A Daniel J. Kingsbury %A Marisa Klein-Gitelman %A Erica F. Lawson %A Suzanne C. Li %A Daniel J. Lovell %A Thomas Mason %A Deborah McCurdy %A Eyal Muscal %A Lorien Nassi %A Egla Rabinovich %A Andreas Reiff %A Margalit Rosenkranz %A Kenneth N. Schikler %A Nora G. Singer %A Steven Spalding %A Anne M. Stevens %A David A. Cabral %T Increased Sensitivity of the European Medicines Agency Algorithm for Classification of Childhood Granulomatosis with Polyangiitis %D 2012 %R 10.3899/jrheum.111352 %J The Journal of Rheumatology %P jrheum.111352 %X 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. %U https://www.jrheum.org/content/jrheum/early/2012/05/14/jrheum.111352.full.pdf