Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood.

Journal Article (Journal Article)

OBJECTIVE: 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)-associated vasculitides (AAVs), and to describe the interval to diagnosis, presenting features, and initial treatment for WG. METHODS: Eligible patients had been diagnosed by site rheumatologists (termed the "MD diagnosis") 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-classified WG patients. RESULTS: MD diagnoses of 117 patients (82 of whom were female) were WG (n = 76), microscopic polyangiitis (n = 17), ANCA-positive pauci-immune glomerulonephritis (n = 5), Churg-Strauss 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-classified WG patients, the median age at diagnosis was 14.2 years (range 4-17 years), and the median interval from symptom onset to diagnosis was 2.7 months (range 0-49 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-four 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). CONCLUSION: 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.

Full Text

Duke Authors

Cited Authors

  • Cabral, DA; Uribe, AG; Benseler, S; O'Neil, KM; Hashkes, PJ; Higgins, G; Zeft, AS; Lovell, DJ; Kingsbury, DJ; Stevens, A; McCurdy, D; Chira, P; Abramson, L; Arkachaisri, T; Campillo, S; Eberhard, A; Hersh, AO; Huber, AM; Kim, S; Klein-Gitelman, M; Levy, DM; Li, SC; Mason, T; Dewitt, EM; Muscal, E; Nassi, L; Reiff, A; Schikler, K; Singer, NG; Wahezi, D; Woodward, A; ARChiVe (A Registry for Childhood Vasculitis: e-entry) Investigators Network,

Published Date

  • November 2009

Published In

Volume / Issue

  • 60 / 11

Start / End Page

  • 3413 - 3424

PubMed ID

  • 19877069

International Standard Serial Number (ISSN)

  • 0004-3591

Digital Object Identifier (DOI)

  • 10.1002/art.24876


  • eng

Conference Location

  • United States