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)

作者：Morishita Kimberly; Li Suzanne C; Muscal Eyal; Spalding Steven; Guzman Jaime; Uribe America; Abramson Leslie; Baszis Kevin; Benseler Susanne; Bowyer Suzanne; Campillo Sarah; Chira Peter; Hersh Aimee O; Higgins Gloria; Eberhard Anne; Ede Kaleo; Imundo Lisa; Jung Lawrence; Kim Susan; King**ury Daniel J; Klein Gitelman Marisa; Lawson Erica F; Lovell Daniel J; Mason Thomas; McCurdy Deborah; Nanda Kabita; Nassi Loren; O' Neil Kathleen M; Rabinovich Egla

来源：Journal of Rheumatology, 2012, 39(5): 1088-1094.

DOI：10.3899/jrheum.111030

• ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES
• VASCULITIS
• ANCA
• WEGENER GRANULOMATOSIS

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%26apos;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). %26lt;br%26gt;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%26apos;s rank correlation coefficient (r(s)) was used to test the strength of association between BVAS v.3 and PGA, treatment decision, and ESR. %26lt;br%26gt;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, r(s) = 0.521 (95% CI 0.393 to 0.629) with treatment decision, and r(s) = 0.403 (95% Cl 0.253 to 0.533) with ESR. %26lt;br%26gt;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. (First Release Feb 15 2012; J Rheumatol 2012;39:1088-94; doi:10.3899/jrheum.111030)

• 出版日期2012-5