Increased Sensitivity of the European Medicines Agency Algorithm for Classification of Childhood Granulomatosis with Polyangiitis.

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 pediatr...

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Publicado en:Journal of Rheumatology Vol. 39; no. 8; pp. 1687 - 1698
Autores principales: Uribe AG, Huber AM, Kim S, O'Neil KM, Wahezi DM, Abramson L, Baszis K, Benseler SM, Bowyer SL, Campillo S, Chira P, Hersh AO, Higgins GC, Eberhard A, Ede K, Imundo LF, Jung L, Kingsbury DJ, Klein-Gitelman M, Lawson EF
Formato: research tables/charts Journal Article
Publicado: Journal of Rheumatology Publishing Co. Ltd. Aug2012
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Aug2012
      vid: 39
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      pub: Journal of Rheumatology Publishing Co. Ltd.
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        2011667409
        10.3899/jrheum.111352
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        atl: Increased Sensitivity of the European Medicines Agency Algorithm for Classification of Childhood Granulomatosis with Polyangiitis.
      aug:
        au:
          Uribe AG
          Huber AM
          Kim S
          O'Neil KM
          Wahezi DM
          Abramson L
          Baszis K
          Benseler SM
          Bowyer SL
          Campillo S
          Chira P
          Hersh AO
          Higgins GC
          Eberhard A
          Ede K
          Imundo LF
          Jung L
          Kingsbury DJ
          Klein-Gitelman M
          Lawson EF
      sug:
        subj:
          Vasculitis Classification
          Granulomatosis with Polyangiitis Classification
          Adolescence
          Child
          Confidence Intervals
          Descriptive Statistics
          Discriminant Validity
          Female
          Funding Source
          Human
          kappa Statistic
          Male
          Record Review
          Retrospective Design
          Sensitivity and Specificity
          Adolescent: 13-18 years
          Child: 6-12 years
          Female
          Male
      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.
      pubtype: Academic Journal
      doctype:
        research
        tables/charts
        Journal Article
      ougenre: Article
    language: English
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