{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T07:27:45Z","timestamp":1780471665110,"version":"3.54.1"},"reference-count":20,"publisher":"The Journal of Rheumatology","issue":"8","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Rheumatol"],"published-print":{"date-parts":[[2012,8]]},"abstract":"<jats:sec><jats:title>Objective.<\/jats:title><jats:p>Granulomatosis with polyangiitis (Wegener\u2019s; 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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods.<\/jats:title><jats:p>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).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results.<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion.<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec>","DOI":"10.3899\/jrheum.111352","type":"journal-article","created":{"date-parts":[[2012,5,16]],"date-time":"2012-05-16T00:34:02Z","timestamp":1337128442000},"page":"1687-1697","source":"Crossref","is-referenced-by-count":38,"title":["Increased Sensitivity of the European Medicines Agency Algorithm for Classification of Childhood Granulomatosis with Polyangiitis"],"prefix":"10.3899","volume":"39","author":[{"given":"AM\u00c9RICA G.","family":"URIBE","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"ADAM M.","family":"HUBER","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"SUSAN","family":"KIM","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"KATHLEEN 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