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

David A. Cabral, América G. Uribe, Susanne Benseler, Kathleen M. O'Neil, Philip J. Hashkes, Gloria Higgins, Andrew S. Zeft, Daniel J. Lovell, Daniel J. Kingsbury, Anne Stevens, Deborah McCurdy, Peter Chira, Leslie Abramson, Thaschawee Arkachaisri, Sarah Campillo, Anne Eberhard, Aimee O. Hersh, Adam M. Huber, Susan Kim, Marisa Klein-GitelmanDeborah M. Levy, Suzanne C. Li, Thomas Mason, Esi Morgan DeWitt, Eyal Muscal, Lorien Nassi, Andreas Reiff, Kenneth Schikler, Nora G. Singer, Dawn Wahezi, Amy Woodward, Victor Espinosa, Jaime Guzman, Kristin Houghton, Peter Malleson, Ross Petty, Lori Tucker, Stuart Turvey, Elizabeth B. Brooks, Mary Lesko, Fatma Dedeoglu, Robert Fuhlbrigge, Melissa Hazen, Mary Beth Son, Robert Sundel, Diane Brown, Bracha Shaham, Ana Cabrera, Norman T. Ilowite, Raphael Hirsh, Daniel Kietz, Paul Rosen, Margalit Rosenkrank, Kathryn Torok, Lauren Pachman, Aisha Ali, Hermine Brunner, Thomas Griffin, Alexi Grom, Anne Johnson, Steven Spalding, Deborah Bork, Stacy Ardoin, Egla Rabinovich, Laura Schanberg, Rhonda Wilder, Bianca A. Lang, Suzanne Ramsey, Elizabeth Stringer, Aleasha Warner, Kathleen Haines, Yukiko Kimura, Jennifer Weiss, Imelda Balboni, Reuven Bromberg, Michal Cidon, Jennifer Frankovich, Dana Gerstbacher, Joyce J. Hsu, Tzielan Lee, Jane L. Park, Christy Sandborg, Steven Song, Ann Reed, Gaëlle Chédeville, Ciarán Duffy, Karen Duffy, Rosie Scuccimarri, Michele Gibbon, Andrew Eichenfield, Lisa Imundo, Candido Batres, Suzanne Bowyer, Susan Ballinger, Thomas Klausmeier, Andrea Hudgins, Helen Emery, Kristin Hayward, Christi Inman, Sarah Ringold, Elizabeth Shaw, Troy Torgerson, Jennifer Turner, Carol Wallace, Jennifer Wargula, Sarah Halford, Barry L. Myones, Marilynn Punaro, Virginia Pascual, Emily von Scheven, Adrienne Michels, Michael Henrickson, James Jarvis, John Bonsack, Sampath Prahalad

Research output: Contribution to journalArticlepeer-review

157 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)3413-3424
Number of pages12
JournalArthritis and rheumatism
Issue number11
StatePublished - Nov 2009

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology
  • Pharmacology (medical)


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