TY - JOUR
T1 - Increased sensitivity of the European Medicines Agency algorithm for classification of childhood granulomatosis with polyangiitis
AU - Uribe, América G.
AU - Huber, Adam M.
AU - Kim, Susan
AU - O'Neil, Kathleen M.
AU - Wahezi, Dawn M.
AU - Abramson, Leslie
AU - Baszis, Kevin
AU - Benseler, Susanne M.
AU - Bowyer, Suzanne L.
AU - Campillo, Sarah
AU - Chira, Peter
AU - Hersh, Aimee O.
AU - Higgins, Gloria C.
AU - Eberhard, Anne
AU - Ede, Kaleo
AU - Imundo, Lisa F.
AU - Jung, Lawrence
AU - Kingsbury, Daniel J.
AU - Klein-Gitelman, Marisa
AU - Lawson, Erica F.
AU - Li, Suzanne C.
AU - Lovell, Daniel J.
AU - Mason, Thomas
AU - McCurdy, Deborah
AU - Muscal, Eyal
AU - Nassi, Lorien
AU - Rabinovich, Egla
AU - Reiff, Andreas
AU - Rosenkranz, Margalit
AU - Schikler, Kenneth N.
AU - Singer, Nora G.
AU - Spalding, Steven
AU - Stevens, Anne M.
AU - Cabral, David A.
PY - 2012/8
Y1 - 2012/8
N2 - Objective. Granulomatosis with polyangiitis (Wegener's; GPA) and other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare in childhood and are sometimes difficult to discriminate. We compared use of adult-derived classification schemes for GPA against validated pediatric criteria in the ARChiVe (A Registry for Childhood Vasculitis e-entry) cohort, a Childhood Arthritis and Rheumatology Research Alliance initiative. Methods. Time-of-diagnosis data for children with physician (MD) diagnosis of AAV and unclassified vasculitis (UCV) from 33 US/Canadian centers were analyzed. The European Medicines Agency (EMA) classification algorithm and European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society (EULAR/PRINTO/ PRES) and American College of Rheumatology (ACR) criteria for GPA were applied to all patients. Sensitivity and specificity were calculated (MD-diagnosis as reference). Results. MD-diagnoses for 155 children were 100 GPA, 25 microscopic polyangiitis (MPA), 6 ANCA-positive pauciimmune glomerulonephritis, 3 Churg-Strauss syndrome, and 21 UCV. Of these, 114 had GPA as defined by EMA, 98 by EULAR/PRINTO/PRES, and 87 by ACR. Fourteen patients were identified as GPA by EULAR/PRINTO/PRES but not by ACR; 3 were identified as GPA by ACR but not EULAR/PRINTO/PRES. Using the EMA algorithm, 135 (87%) children were classifiable. The sensitivity of the EMA algorithm, the EULAR/PRINTO/PRES, and ACR criteria for classifying GPA was 90%, 77%, and 69%, respectively, with specificities of 56%, 62%, and 67%. The relatively poor sensitivity of the 2 criteria related to their inability to discriminate patients with MPA. Conclusion. EULAR/PRINTO/PRES was more sensitive than ACR criteria in classifying pediatric GPA. Neither classification system has criteria for MPA; therefore usefulness in discriminating patients in ARChiVe was limited. Even when using the most sensitive EMA algorithm, many children remained unclassified. The Journal of Rheumatology
AB - Objective. Granulomatosis with polyangiitis (Wegener's; GPA) and other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare in childhood and are sometimes difficult to discriminate. We compared use of adult-derived classification schemes for GPA against validated pediatric criteria in the ARChiVe (A Registry for Childhood Vasculitis e-entry) cohort, a Childhood Arthritis and Rheumatology Research Alliance initiative. Methods. Time-of-diagnosis data for children with physician (MD) diagnosis of AAV and unclassified vasculitis (UCV) from 33 US/Canadian centers were analyzed. The European Medicines Agency (EMA) classification algorithm and European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society (EULAR/PRINTO/ PRES) and American College of Rheumatology (ACR) criteria for GPA were applied to all patients. Sensitivity and specificity were calculated (MD-diagnosis as reference). Results. MD-diagnoses for 155 children were 100 GPA, 25 microscopic polyangiitis (MPA), 6 ANCA-positive pauciimmune glomerulonephritis, 3 Churg-Strauss syndrome, and 21 UCV. Of these, 114 had GPA as defined by EMA, 98 by EULAR/PRINTO/PRES, and 87 by ACR. Fourteen patients were identified as GPA by EULAR/PRINTO/PRES but not by ACR; 3 were identified as GPA by ACR but not EULAR/PRINTO/PRES. Using the EMA algorithm, 135 (87%) children were classifiable. The sensitivity of the EMA algorithm, the EULAR/PRINTO/PRES, and ACR criteria for classifying GPA was 90%, 77%, and 69%, respectively, with specificities of 56%, 62%, and 67%. The relatively poor sensitivity of the 2 criteria related to their inability to discriminate patients with MPA. Conclusion. EULAR/PRINTO/PRES was more sensitive than ACR criteria in classifying pediatric GPA. Neither classification system has criteria for MPA; therefore usefulness in discriminating patients in ARChiVe was limited. Even when using the most sensitive EMA algorithm, many children remained unclassified. The Journal of Rheumatology
KW - Classification criteria
KW - Granulomatosis
KW - Pediatric rheumatology
KW - Polyangiitis
KW - Vasculitis
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U2 - 10.3899/jrheum.111352
DO - 10.3899/jrheum.111352
M3 - Article
C2 - 22589257
AN - SCOPUS:84864532703
SN - 0315-162X
VL - 39
SP - 1687
EP - 1697
JO - Journal of Rheumatology
JF - Journal of Rheumatology
IS - 8
ER -