{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,3]],"date-time":"2026-11-03T15:00:58Z","timestamp":1793718058644,"version":"4.6.1"},"reference-count":27,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2009,10,29]],"date-time":"2009-10-29T00:00:00Z","timestamp":1256774400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis &amp; Rheumatism"],"published-print":{"date-parts":[[2009,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>To compare the criteria for Wegener's granulomatosis (WG) of the American College of Rheumatology (ACR) with those of the European League Against Rheumatism\/Pediatric Rheumatology European Society (EULAR\/PRES) in a cohort of children with WG and other antineutrophil cytoplasmic antibody (ANCA)\u2013associated vasculitides (AAVs), and to describe the interval to diagnosis, presenting features, and initial treatment for WG.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Eligible patients had been diagnosed by site rheumatologists (termed the \u201cMD diagnosis\u201d) since 2004. This diagnosis was used as a reference standard for sensitivity and specificity testing of the 2 WG classification criteria. Descriptive analyses were confined to ACR\u2010classified WG patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>MD diagnoses of 117 patients (82 of whom were female) were WG (n = 76), microscopic polyangiitis (n = 17), ANCA\u2010positive pauci\u2010immune glomerulonephritis (n = 5), Churg\u2010Strauss syndrome (n = 2), and unclassified vasculitis (n = 17). The sensitivities of the ACR and EULAR\/PRES classification criteria for WG among the spectrum of AAVs were 68.4% and 73.6%, respectively, and the specificities were 68.3% and 73.2%, respectively. Two more children were identified as having WG by the EULAR\/PRES criteria than by the ACR criteria. For the 65 ACR\u2010classified WG patients, the median age at diagnosis was 14.2 years (range 4\u201317 years), and the median interval from symptom onset to diagnosis was 2.7 months (range 0\u201349 months). The most frequent presenting features by organ system were constitutional (89.2%), pulmonary (80.0%), ear, nose, and throat (80.0%), and renal (75.4%). Fifty\u2010four patients (83.1%) commenced treatment with the combination of corticosteroids and cyclophosphamide, with widely varying regimens; the remainder received methotrexate alone (n = 1), corticosteroids alone (n = 4), or a combination (n = 6).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The EULAR\/PRES criteria minimally improved diagnostic sensitivity and specificity for WG among a narrow spectrum of children with AAVs. Diagnostic delays may result from poor characterization of childhood WG. Initial therapy varied considerably among participating centers.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.24876","type":"journal-article","created":{"date-parts":[[2009,10,29]],"date-time":"2009-10-29T21:16:37Z","timestamp":1256850997000},"page":"3413-3424","source":"Crossref","is-referenced-by-count":172,"title":["Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood"],"prefix":"10.1002","volume":"60","author":[{"given":"David A.","family":"Cabral","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Am\u00e9rica G.","family":"Uribe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanne","family":"Benseler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathleen 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