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.

Relative Performance of Two Validated Classification Systems for Wegener's Granulomatosis among Patients with ANCA-Associated Vasculitis (AAV) in a Registry for Children with Vasculitis: e-entry (ARChiVe).

Uribe3,  America G., Cabral3,  David A., Morishita2,  Kimberly, Abramson38,  Leslie S., Adams6,  Matthew D., Baszis30,  Kevin W., Benseler32,  Susanne M.

Baylor College of Medicine, Houston, TX
Childrens Hospital of NY, New York, NY
Childrens Hospital of Pittsburgh, Pittsburgh, PA
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 Ctr, Halifax, NS, Canada
Joseph M Sanzari Children's Hospital, Short Hills, NJ
Legacy Emanuel Children's Hosp, Portland, OR
Mattel Children's UCLA, Los Angeles, CA
BC Children's Hospital
Mayo Clinic Rochester, Rochester, MN
MetroHealth Medical Center, Cleveland, OH
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
Riley Children's Hospital, Nashville, TN
Robert Wood Johnson Medical School, Metuchen, NJ
Schneider Children's Hospital, New Hyde Park, NY
BC Children's Hospital, Vancouver, BC, Canada
St. Louis Children's Hospital, St Louis, MO
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
Children's Hospital at Montefiore, Bronx, NY
Children's Hospital Med Ctr, Akron, OH
Children's Hospital of Michigan, Huntington Woods, MI
Childrens Hosp LA MS60, Los Angeles, CA
Childrens Hospital Boston, Boston, MA
Childrens Hospital Medical Center, Cincinnati, OH


The European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society (EULAR/PRINTO/PRES) classification criteria for Wegener's Granulomatosis (WG), proposed to be more applicable to children than American College of Rheumatology (ACR) criteria, were recently validated in Ankara. The European Medicines Agency (EMEA) classification algorithm aimed to more uniquely classify related small to medium size vessel vasculitides was also recently validated in adults.


To test the relative performance of the EULAR/PRINTO/PRES classification criteria for WG versus the ACR, applied in a cohort of children with AAV, and concurrently test the EMEA algorithm.


Data from time of diagnosis on children with diagnosed AAV by pediatric rheumatologists (MD-diagnosis) have been contributed to ARChiVe from 37 US/Canadian centers since 2004. EULAR/PRINTO/PRES and ACR criteria for WG and the EMEA algorithm were applied to all patients. Sensitivity and specificity for the EULAR/PRINTO/PRES and ACR classification criteria, and the EMEA algorithm were calculated (MD-diagnosis as reference standard). Cases where classification of a patient by EULAR/PRINTO/PRES versus ACR differed were described.


MD-diagnoses for 155 patients (105 female) were: 100 WG, 25 Microscopic polyangiitis, 6 ANCA-positive pauci-immune glomerulonephritis, 3 Churg-Strauss syndrome, and 21 unclassified vasculitis. Among these patients, 87 had WG as defined by ACR, 98 by EULAR/PRINTO/PRES, and 113 by the EMEA algorithm. Respectively, the sensitivity of the ACR and EULAR/PRINTO/PRES criteria, and the EMEA algorithm for classifying WG patients in the spectrum of patients in an AAV cohort was 69%, 77%, and 90%; and specificity 67%, 62% and 60%. There were 17 patients in whom WG classification by EULAR/PRINTO/PRES versus ACR criteria differed. 14 were identified as WG by EULAR/PRINTO/PRES but not by ACR criteria: features that enabled classification were sinus involvement, ANCA positivity, subglottic-tracheal-endobronchial stenosis, or significant proteinuria. Conversely, 3 cases with WG were classified by ACR but not by EULAR/PRINTO/PRES: 2 patients had nasal-sinus involvement and granulomatous vasculitis on biopsy, and 1 patient had lung and renal involvement; all three had negative ANCA serology. Using the EMEA algorithm, 134 children could be classified as a named AAV category; 21 (14%) patients could not be classified.


The EULAR/PRINTO/PRES criteria are more sensitive than the ACR criteria in classifying pediatric WG. Performance of both criteria were limited when applied to a cohort that includes patients with MPA for which neither classification systems have established criteria, unlike the most sensitive EMEA classification algorithm which specifically aims to uniquely classify all patients. However, even when using the EMEA algorithm many children diagnosed with AAV remained unclassified.

To cite this abstract, please use the following information:
Uribe, America G., Cabral, David A., Morishita, Kimberly, Abramson, Leslie S., Adams, Matthew D., Baszis, Kevin W., et al; Relative Performance of Two Validated Classification Systems for Wegener's Granulomatosis among Patients with ANCA-Associated Vasculitis (AAV) in a Registry for Children with Vasculitis: e-entry (ARChiVe). [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1691
DOI: 10.1002/art.29456

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