Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood
Article first published online: 29 OCT 2009
Copyright © 2009 by the American College of Rheumatology
Arthritis & Rheumatism
Volume 60, Issue 11, pages 3413–3424, November 2009
How to Cite
Cabral, D. A., Uribe, A. G., Benseler, S., O'Neil, K. M., Hashkes, P. J., Higgins, G., Zeft, A. S., Lovell, D. J., Kingsbury, D. J., Stevens, A., McCurdy, D., Chira, P., Abramson, L., Arkachaisri, T., Campillo, S., Eberhard, A., Hersh, A. O., Huber, A. M., Kim, S., Klein-Gitelman, M., Levy, D. M., Li, S. C., Mason, T., DeWitt, E. M., Muscal, E., Nassi, L., Reiff, A., Schikler, K., Singer, N. G., Wahezi, D. and Woodward, A. (2009), Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood. Arthritis & Rheumatism, 60: 3413–3424. doi: 10.1002/art.24876
- Issue published online: 29 OCT 2009
- Article first published online: 29 OCT 2009
- Manuscript Accepted: 11 JUL 2009
- Manuscript Received: 2 JAN 2009
- Vasculitis Foundation (formerly known as the Wegener's Granulomatosis Association)
- British Columbia Children's Hospital Foundation Telethon Grant
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.
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.
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).
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.