Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

Describing the Performance of the Birmingham Vasculitis Activity Score (BVAS) at Diagnosis for Children with ANCA-Associated Vasculitis (AAV) in A Registry for Children with Vasculitis (ARChiVe).

Morishita2,  Kimberly A., Uribe2,  America, Guzman2,  Jaime, Abramson37,  Leslie S., Adams8,  Matthew D., Benseler31,  Susanne M., Brooks4,  Elizabeth B.

Baylor College of Medicine, Houston, TX
Childrens Hosp LA MS60, Los Angeles, CA
Childrens Hospital Boston, Boston, MA
Childrens Hospital Medical Center, Cincinnati, OH
Childrens Hospital of NY, New York, NY
Childrens Med Ctr Med Schl GA, Augusta, GA
Childrens Mem Hosp/NW Univ, Chicago, IL
Corner Children's Hospital, Chicago, IL
Duke University Medical Center, Durham, NC
IWK Health Centre, Halifax, NS, Canada
Legacy Emanuel Children's Hosp, Portland, OR
BC Children's Hospital
Mattel Children's UCLA, Los Angeles, CA
Mayo Clinic Rochester, Rochester, MN
Montreal Children's Hospital, Montreal, QC, Canada
Nationwide Childrens Hosp, Columbus, OH
OU Health Science Center, Oklahoma City, OK
Pediatrics, U. of Washington, Seattle, WA
Phoenix Children's Hospital, Phoenix, AZ
Robert Wood Johnson Medical School, Metuchen, NJ
Schneider Children's Hospital, New Hyde Park, NY
St Louis Childrens Hosp, St Louis, MO
BC Children's Hospital, Vancouver, BC, Canada
Stanford Univ Schl of Med, Stanford, CA
The Hospital for Sick Children, Toronto, ON, Canada
TX Scottish Rite Hosp, Dallas, TX
UCSF, San Francisco, CA
University of Florida, Orlando, FL
University of Louisville SOM, Louisville, KY
University of Utah, Salt Lake City, UT
University of Vermont, Morrisville, VT
Vanderbilt Univ. School of Medicine, Nashville, TN
Case Western, Beachwood, OH
Children's Hospital at Montefiore, Bronx, NY
Children's Hospital Med Ctr, Akron, OH
Children's Hospital of Boston, Short Hills, NJ
Children's Hospital of Michigan, Huntington Woods, MI
Children's Hospital of Pittsburg, Pittsburgh, PA


There are no validated paediatric assessment tools for measuring disease activity in chronic vasculitis. The BVAS, a valid and reliable assessment tool for measuring disease activity in adults with vasculitis, may not necessarily capture the clinical features or components of disease activity assessment that might be unique to, or more characteristic of, the growing and developing child. In adult vasculitis, BVAS correlates highly (rho = 0.91) with physician's global assessment of disease activity (PGA).


To describe the performance of BVAS applied at the time of diagnosis in children with AAV in relationship to PGA.


Eligible patients were those diagnosed since 2004 with an AAV, according to expert site rheumatologist (MD-diagnosis) at 37 ARChiVe centers. Data collection items in the registry included all elements of BVAS and a PGA for patients at the time of diagnosis. After BVAS items were extracted and individual patient scores calculated, the association between BVAS (version 3) and PGA scores was assessed using the Spearman rank correlation coefficient. The statistical analysis was performed using SPSS statistical software.


155 patients with an AAV were recruited into the ARChiVe cohort between January 2004 and March 2010. Five patients were excluded from analysis because data was insufficient to score BVAS. The MD-diagnosis of the remaining 150 patients were Wegener's granulomatosis (n = 98), microscopic polyangiitis (n = 25), Churg-Strauss syndrome (n = 3), ANCA-positive pauci-immune glomerulonephritis (n = 5), and unclassified vasculitis (n = 19). The correlation between BVAS and PGA scores was weak in children (rho = 0.379, 95% CI 0.233 to 0.509, p < 0.0001) (Figure).


The poor correlation of BVAS with PGA suggests either that BVAS requires significant paediatric specific modification, or, that there is no uniformity or standards of disease activity assessment among paediatric rheumatologists. The tool may also perform better measuring change of activity between visits rather than quantifying disease activity at diagnosis. Development of a standardized tool to enable clinical trials in children to improve outcomes is essential.

To cite this abstract, please use the following information:
Morishita, Kimberly A., Uribe, America, Guzman, Jaime, Abramson, Leslie S., Adams, Matthew D., Benseler, Susanne M., et al; Describing the Performance of the Birmingham Vasculitis Activity Score (BVAS) at Diagnosis for Children with ANCA-Associated Vasculitis (AAV) in A Registry for Children with Vasculitis (ARChiVe). [abstract]. Arthritis Rheum 2010;62 Suppl 10 :2017
DOI: 10.1002/art.29782

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