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

Publication ,  Journal Article
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 ...
Published in: J Rheumatol
August 2012

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.

Duke Scholars

Published In

J Rheumatol

DOI

EISSN

1499-2752

Publication Date

August 2012

Volume

39

Issue

8

Start / End Page

1687 / 1697

Location

Canada

Related Subject Headings

  • Sensitivity and Specificity
  • Registries
  • Microscopic Polyangiitis
  • Male
  • Humans
  • Granulomatosis with Polyangiitis
  • Female
  • Diagnosis, Differential
  • Churg-Strauss Syndrome
  • Child
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Uribe, A. G., Huber, A. M., Kim, S., O’Neil, K. M., Wahezi, D. M., Abramson, L., … ARegistry for Children with Vasculitis e-entry (ARChiVe) Network, . (2012). Increased sensitivity of the European medicines agency algorithm for classification of childhood granulomatosis with polyangiitis. J Rheumatol, 39(8), 1687–1697. https://doi.org/10.3899/jrheum.111352
Uribe, América G., Adam M. Huber, Susan Kim, Kathleen M. O’Neil, Dawn M. Wahezi, Leslie Abramson, Kevin Baszis, et al. “Increased sensitivity of the European medicines agency algorithm for classification of childhood granulomatosis with polyangiitis.J Rheumatol 39, no. 8 (August 2012): 1687–97. https://doi.org/10.3899/jrheum.111352.
Uribe AG, Huber AM, Kim S, O’Neil KM, Wahezi DM, Abramson L, et al. Increased sensitivity of the European medicines agency algorithm for classification of childhood granulomatosis with polyangiitis. J Rheumatol. 2012 Aug;39(8):1687–97.
Uribe, América G., et al. “Increased sensitivity of the European medicines agency algorithm for classification of childhood granulomatosis with polyangiitis.J Rheumatol, vol. 39, no. 8, Aug. 2012, pp. 1687–97. Pubmed, doi:10.3899/jrheum.111352.
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, Li SC, Lovell DJ, Mason T, McCurdy D, Muscal E, Nassi L, Rabinovich E, Reiff A, Rosenkranz M, Schikler KN, Singer NG, Spalding S, Stevens AM, Cabral DA, ARegistry for Children with Vasculitis e-entry (ARChiVe) Network. Increased sensitivity of the European medicines agency algorithm for classification of childhood granulomatosis with polyangiitis. J Rheumatol. 2012 Aug;39(8):1687–1697.

Published In

J Rheumatol

DOI

EISSN

1499-2752

Publication Date

August 2012

Volume

39

Issue

8

Start / End Page

1687 / 1697

Location

Canada

Related Subject Headings

  • Sensitivity and Specificity
  • Registries
  • Microscopic Polyangiitis
  • Male
  • Humans
  • Granulomatosis with Polyangiitis
  • Female
  • Diagnosis, Differential
  • Churg-Strauss Syndrome
  • Child