1. Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood
- Author
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Raphael Hirsh, Aisha Ali, Susanne M. Benseler, Robert P. Sundel, Karen Watanabe Duffy, Elizabeth A. Shaw, Jane L. Park, Ciarán M. Duffy, Rosie Scuccimarri, Peter Chira, Laura E. Schanberg, Melissa M. Hazen, Christi J. Inman, Deborah Bork, Amy Woodward, Suzanne E. Ramsey, Elizabeth B. Brooks, Yukiko Kimura, Dawn M. Wahezi, Jennifer Frankovich, Kristin Houghton, Andrew Zeft, Dana Gerstbacher, Robert C. Fuhlbrigge, James N. Jarvis, Susan H. Ballinger, Candido Batres, Suzanne C. Li, Bracha Shaham, Anne M. Stevens, Jennifer Turner, Deborah McCurdy, Esi Morgan DeWitt, Ana Cabrera, Margalit Rosenkrank, Reuven Bromberg, Lisa Imundo, John Bonsack, Andrea Hudgins, David A. Cabral, A. Grom, Kenneth N. Schikler, Lorien Nassi, Sarah Ringold, Bianca A. Lang, Daniel J. Kingsbury, Aleasha Warner, Rhonda Wilder, Michael Henrickson, Fatma Dedeoglu, Gaëlle Chédeville, Troy R. Torgerson, Suzanne L. Bowyer, Mary Beth F. Son, Marilynn Punaro, Daniel Kietz, Diane E. Brown, Imelda Balboni, Helen Emery, Sarah Halford, Michal Cidon, Marisa S. Klein-Gitelman, Tzielan Lee, Steven J. Spalding, Anne Eberhard, Anne Johnson, Leslie Abramson, Adrienne Michels, Kristin Hayward, Paul Rosen, Michele Gibbon, Thomas G. Mason, Andreas Reiff, Peter N. Malleson, Lauren M. Pachman, Deborah M. Levy, Victor Espinosa, Kathleen A. Haines, Christy Sandborg, Carol A. Wallace, Adam M. Huber, Hermine I. Brunner, Lori B. Tucker, Steven J. Song, Philip J. Hashkes, S Prahalad, Stacy P. Ardoin, Thomas A. Griffin, Elizabeth Stringer, Virginia Pascual, Gloria C. Higgins, América G. Uribe, Jennifer Wargula, Stuart E. Turvey, Kathleen M. O'Neil, Jaime Guzman, Andrew H. Eichenfield, Thaschawee Arkachaisri, Norman T. Ilowite, Sarah Campillo, Ann M. Reed, Aimee O. Hersh, J Weiss, Susan Kim, Barry L. Myones, Mary Lesko, Emily von Scheven, Joyce J. Hsu, Nora G. Singer, Eyal Muscal, Thomas Klausmeier, Daniel J. Lovell, Egla Rabinovich, Kathryn S. Torok, and Ross E. Petty
- Subjects
Male ,Vasculitis ,medicine.medical_specialty ,Systemic disease ,Adolescent ,Immunology ,Microscopic Polyangiitis ,Pilot Projects ,Churg-Strauss Syndrome ,Sensitivity and Specificity ,Cohort Studies ,Diagnosis, Differential ,Glomerulonephritis ,Rheumatology ,Adrenal Cortex Hormones ,Internal medicine ,medicine ,Humans ,Immunology and Allergy ,Pharmacology (medical) ,Child ,skin and connective tissue diseases ,Cyclophosphamide ,Societies, Medical ,Anti-neutrophil cytoplasmic antibody ,business.industry ,Granulomatosis with Polyangiitis ,Reference Standards ,medicine.disease ,United States ,Surgery ,Europe ,Methotrexate ,Child, Preschool ,Cohort ,Female ,business ,Microscopic polyangiitis ,Rheumatism ,Cohort study - Abstract
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.
- Published
- 2009
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