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

Publication ,  Journal Article
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 ...
Published in: Arthritis Rheum
November 2009

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.

Duke Scholars

Published In

Arthritis Rheum

DOI

ISSN

0004-3591

Publication Date

November 2009

Volume

60

Issue

11

Start / End Page

3413 / 3424

Location

United States

Related Subject Headings

  • Vasculitis
  • United States
  • Societies, Medical
  • Sensitivity and Specificity
  • Reference Standards
  • Pilot Projects
  • Microscopic Polyangiitis
  • Methotrexate
  • Male
  • Humans
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Cabral, D. A., Uribe, A. G., Benseler, S., O’Neil, K. M., Hashkes, P. J., Higgins, G., … ARChiVe (A Registry for Childhood Vasculitis: e-entry) Investigators Network, . (2009). Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood. Arthritis Rheum, 60(11), 3413–3424. https://doi.org/10.1002/art.24876
Cabral, David A., América G. Uribe, Susanne Benseler, Kathleen M. O’Neil, Philip J. Hashkes, Gloria Higgins, Andrew S. Zeft, et al. “Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood.Arthritis Rheum 60, no. 11 (November 2009): 3413–24. https://doi.org/10.1002/art.24876.
Cabral DA, Uribe AG, Benseler S, O’Neil KM, Hashkes PJ, Higgins G, et al. Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood. Arthritis Rheum. 2009 Nov;60(11):3413–24.
Cabral, David A., et al. “Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood.Arthritis Rheum, vol. 60, no. 11, Nov. 2009, pp. 3413–24. Pubmed, doi:10.1002/art.24876.
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. Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood. Arthritis Rheum. 2009 Nov;60(11):3413–3424.
Journal cover image

Published In

Arthritis Rheum

DOI

ISSN

0004-3591

Publication Date

November 2009

Volume

60

Issue

11

Start / End Page

3413 / 3424

Location

United States

Related Subject Headings

  • Vasculitis
  • United States
  • Societies, Medical
  • Sensitivity and Specificity
  • Reference Standards
  • Pilot Projects
  • Microscopic Polyangiitis
  • Methotrexate
  • Male
  • Humans