Details

Autor(en) / Beteiligte

• 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

Titel

Increased sensitivity of the European medicines agency algorithm for classification of childhood granulomatosis with polyangiitis

Ist Teil von

• Journal of rheumatology, 2012-08, Vol.39 (8), p.1687-1697

Ort / Verlag

Canada

Erscheinungsjahr

2012

Quelle

MEDLINE

Beschreibungen/Notizen

• 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. 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). 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. 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.