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


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.

DOI: 10.1002/art.24876
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@article{Cabral2009ClassificationPA, title={Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood.}, author={David A Cabral and Am{\'e}rica G Uribe and Susanne Benseler and Kathleen M O'Neil and Philip J Hashkes and Gloria Higgins and Andrew S Zeft and Daniel J Lovell and Daniel J Kingsbury and Anne Stevens and Deborah McCurdy and Peter Chira and Leslie Abramson and Thaschawee Arkachaisri and Sarah Campillo and Anne Eberhard and Aimee O Hersh and Adam M Huber and Susan Kim and Marisa Klein-Gitelman and Deborah M Levy and Suzanne C Li and Thomas Mason and Esi Morgan Dewitt and Eyal Muscal and Lorien Nassi and Andreas Reiff and Kenneth Schikler and Nora G Singer and Dawn Wahezi and Amy Woodward}, journal={Arthritis and rheumatism}, year={2009}, volume={60 11}, pages={3413-24} }