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EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides
  1. S Ozen1,
  2. N Ruperto2,
  3. M J Dillon3,
  4. A Bagga4,
  5. K Barron5,
  6. J C Davin6,
  7. T Kawasaki7,
  8. C Lindsley8,
  9. R E Petty9,
  10. A M Prieur10,
  11. A Ravelli2,
  12. P Woo11
  1. 1Department of Paediatrics, Hacettepe University, Ankara, Turkey
  2. 2IRCCS G Gaslini, Pediatria II, Genova, Italy
  3. 3Paediatric Nephrology, Institute of Child Health and Great Ormond Street Hospital for Sick Children, London, UK
  4. 4Department of Paediatrics, All India Institute of Medical Sciences, New Delhi, India
  5. 5National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, USA
  6. 6Emma Children’s Hospital/Academic Medical Centre, Amsterdam, Netherlands
  7. 7Japan Kawasaki Disease Research Centre, Tokyo, Japan
  8. 8Department of Pediatrics, University of Kansas Medical Center, Kansas, USA
  9. 9Department of Pediatrics, University of British Columbia, Vancouver, Canada
  10. 10Unité d’immunologie, hématology, et rheumatologie pédiatrique, Hôpital Necker Enfants Maladies, Paris, France
  11. 11Rheumatology Department, Great Ormond Street Hospital for Sick Children, London, UK
  1. Correspondence to:
    Professor Dr Seza Ozen
    Department of Paediatrics, Hacettepe University Faculty of Medicine, 06100 Ankara, Turkey; sezaozen{at}


Background: There has been a lack of appropriate classification criteria for vasculitis in children.

Objective: To develop a widely accepted general classification for the vasculitides observed in children and specific and realistic classification criteria for common childhood vasculitides (Henoch-Schönlein purpura (HSP), Kawasaki disease (KD), childhood polyarteritis nodosa (PAN), Wegener’s granulomatosis (WG), and Takayasu arteritis (TA)).

Methods: The project was divided into two phases: (1) the Delphi technique was used to gather opinions from a wide spectrum of paediatric rheumatologists and nephrologists; (2) a consensus conference using nominal group technique was held. Ten international experts, all paediatricians, met for the consensus conference. Agreement of at least 80% of the participants was defined as consensus.

Results: Consensus was reached to base the general working classification for childhood vasculitides on vessel size. The small vessel disease was further subcategorised into “granulomatous” and “non-granulomatous.” Final criteria were developed to classify a child as HSP, KD, childhood PAN, WG, or TA, with changes introduced based on paediatric experience. Mandatory criteria were suggested for all diseases except WG.

Conclusions: It is hoped that the suggested criteria will be widely accepted around the world because of the reliable techniques used and the international and multispecialist composition of the expert group involved.

  • ACR, American College of Rheumatology
  • ANCA, antineutrophil cytoplasmic antibodies
  • ESPN, European Society of Paediatric Nephrology
  • EULAR, European League against Rheumatism
  • HSP, Henoch-Schönlein purpura
  • KD, Kawasaki disease
  • NGT, nominal group techniques
  • PAN, polyarteritis nodosa
  • PReS, Paediatric Rheumatology European Society
  • TA, Takayasu arteritis
  • WG, Wegener’s granulomatosis
  • child
  • vasculitis
  • classification
  • childhood polyarteritis nodosa

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