Increased sensitivity of the European medicines agency algorithm for classification of childhood granulomatosis with polyangiitis.
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Uribe América G, Huber Adam M, Kim Susan, O'Neil Kathleen M, Wahezi Dawn M, Abramson Leslie, Baszis Kevin, Benseler Susanne M, Bowyer Suzanne L, Campillo Sarah, Chira Peter, Hersh Aimee O, Higgins Gloria C, Eberhard Anne, Ede Kaleo, Imundo Lisa F, Jung Lawrence, Kingsbury Daniel J, Klein-Gitelman Marisa, Lawson Erica F, Li Suzanne C, Lovell Daniel J, Mason Thomas, McCurdy Deborah, Muscal Eyal, Nassi Lorien, Rabinovich Egla, Reiff Andreas, Rosenkranz Margalit, Schikler Kenneth N, Singer Nora G, Spalding Steven, Stevens Anne M, Cabral David A, ARegistry for Children with Vasculitis e-entry (ARChiVe) Network
Аннотация
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.