Assessing the performance of the Birmingham vasculitis activity score at diagnosis for Children with antineutrophil cytoplasmic antibody-associated vasculitis in a registry for childhood vasculitis (ARChiVe)

Kimberly Morishita, Suzanne C. Li, Eyal Muscal, Steven Spalding, Jaime Guzman, America Uribe, Leslie Abramson, Kevin Baszis, Susanne Benseler, Suzanne Bowyer, Sarah Campillo, Peter Chira, Aimee O. Hersh, Gloria Higgins, Anne Eberhard, Kaleo Ede, Lisa Imundo, Lawrence Jung, Susan Kim, Daniel J. KingsburyMarisa Klein-Gitelman, Erica F. Lawson, Daniel J. Lovell, Thomas Mason, Deborah McCurdy, Kabita Nanda, Lorien Nassi, Kathleen M. O'Neil, Egla Rabinovich, Suzanne E. Ramsey, Andreas Reiff, Margalit Rosenkranz, Kenneth Schikler, Anne Stevens, Dawn Wahezi, David A. Cabral

Research output: Contribution to journalArticlepeer-review

12 Scopus citations

Abstract

Objective: There are no validated tools for measuring disease activity in pediatric vasculitis. The Birmingham Vasculitis Activity Score (BVAS) is a valid disease activity tool in adult vasculitis. Version 3 (BVAS v.3) correlates well with physician's global assessment (PGA), treatment decision, and C-reactive protein in adults. The utility of BVAS v.3 in pediatric vasculitis is not known. We assessed the association of BVAS v.3 scores with PGA, treatment decision, and erythrocyte sedimentation rate (ESR) at diagnosis in pediatric antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Methods: Children with AAV diagnosed between 2004 and 2010 at all ARChiVe centers were eligible. BVAS v.3 scores were calculated with a standardized online tool (www.vasculitis.org). Spearman's rank correlation coefficient (rs) was used to test the strength of association between BVAS v.3 and PGA, treatment decision, and ESR. Results: A total of 152 patients were included. The physician diagnosis of these patients was predominantly granulomatosis with polyangiitis (n = 99). The median BVAS v.3 score was 18.0 (range 0-40). The BVAS v.3 correlations were rs = 0.379 (95% CI 0.233 to 0.509) with PGA, rs = 0.521 (95% CI 0.393 to 0.629) with treatment decision, and rs = 0.403 (95% CI 0.253 to 0.533) with ESR. Conclusion: Applied to children with AAV, BVAS v.3 had a weak correlation with PGA and moderate correlation with both ESR and treatment decision. Prospective evaluation of BVAS v.3 and/or pediatric-specific modifications to BVAS v.3 may be required before it can be formalized as a disease activity assessment tool in pediatric AAV.

Original languageEnglish (US)
Pages (from-to)1088-1094
Number of pages7
JournalJournal of Rheumatology
Volume39
Issue number5
DOIs
StatePublished - May 2012

Keywords

  • Anca
  • Antineutrophil cytoplasmic antibodies
  • Vasculitis
  • Wegener granulomatosis

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Immunology

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