EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation

Nicolino Ruperto, Seza Ozen, Angela Pistorio, Pavla Dolezalova, Paul Brogan, David A. Cabral, Ruben Cuttica, Raju Khubchandani, Daniel J. Lovell, Kathleen M. O'Neil, Pierre Quartier, Angelo Ravelli, Silvia M. Iusan, Giovanni Filocamo, Claudia Saad Magalhães, Erbil Unsal, Sheila Oliveira, Claudia Bracaglia, Arvind Bagga, Valda StanevichaSilvia Magni Manzoni, Polyxeni Pratsidou, Loredana Lepore, Graciela Espada, Isabelle Kone Paut, Francesco Zulian, Patrizia Barone, Zelal Bircan, Maria Del Rocio Maldonado, Ricardo Russo, Iris Vilca, Kjell Tullus, Rolando Cimaz, Gerd Horneff, Jordi Anton, Stella Garay, Susan Nielsen, Giancarlo Barbano, Alberto Martini

Research output: Contribution to journalArticlepeer-review

Abstract

Objectives: To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria. Methods: The preliminary Vienna 2005 consensus conference, which proposed preliminary criteria for paediatric vasculitides, was followed by a EULAR/PRINTO/PRES - supported validation project divided into three main steps. Step 1: retrospective/prospective web-data collection for HSP, c-PAN, c-WG and c-TA, with age at diagnosis ≤18 years. Step 2: blinded classification by consensus panel of a subgroup of 280 cases (128 difficult cases, 152 randomly selected) enabling expert diagnostic verification. Step 3: Ankara 2008 Consensus Conference and statistical evaluation (sensitivity, specificity, area under the curve, κ-agreement) using as 'gold standard' the final consensus classification or original treating physician diagnosis. Results: A total of 1183/1398 (85%) samples collected were available for analysis: 827 HSP, 150 c-PAN, 60 c-WG, 87 c-TA and 59 c-other. Prevalence, signs/symptoms, laboratory, biopsy and imaging reports were consistent with the clinical picture of the four c-vasculitides. A representative subgroup of 280 patients was blinded to the treating physician diagnosis and classified by a consensus panel, with a κ-agreement of 0.96 for HSP (95% CI 0.84 to 1), 0.88 for c-WG (95% CI 0.76 to 0.99), 0.84 for c-TA (95% CI 0.73 to 0.96) and 0.73 for c-PAN (95% CI 0.62 to 0.84), with an overall κ of 0.79 (95% CI 0.73 to 0.84). Conclusion: EULAR/PRINTO/PRES propose validated classification criteria for HSP, c-PAN, c-WG and c-TA, with substantial/almost perfect agreement with the final consensus classification or original treating physician diagnosis.

Original languageEnglish
Pages (from-to)790-797
Number of pages8
JournalAnnals of the Rheumatic Diseases
Volume69
Issue number5
DOIs
Publication statusPublished - May 2010

ASJC Scopus subject areas

  • Rheumatology
  • Immunology
  • Biochemistry, Genetics and Molecular Biology(all)
  • Immunology and Allergy

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