EULAR/PReS endorsed consensus criteria* for the classification of childhood vasculitides
- S Ozen1,
- N Ruperto2,
- M J Dillon3,
- A Bagga4,
- K Barron5,
- J C Davin6,
- T Kawasaki7,
- C Lindsley8,
- R E Petty9,
- A M Prieur10,
- A Ravelli2,
- P Woo11
- 1Department of Paediatrics, Hacettepe University, Ankara, Turkey
- 2IRCCS G Gaslini, Pediatria II, Genova, Italy
- 3Paediatric Nephrology, Institute of Child Health and Great Ormond Street Hospital for Sick Children, London, UK
- 4Department of Paediatrics, All India Institute of Medical Sciences, New Delhi, India
- 5National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, USA
- 6Emma Children’s Hospital/Academic Medical Centre, Amsterdam, Netherlands
- 7Japan Kawasaki Disease Research Centre, Tokyo, Japan
- 8Department of Pediatrics, University of Kansas Medical Center, Kansas, USA
- 9Department of Pediatrics, University of British Columbia, Vancouver, Canada
- 10Unité d’immunologie, hématology, et rheumatologie pédiatrique, Hôpital Necker Enfants Maladies, Paris, France
- 11Rheumatology Department, Great Ormond Street Hospital for Sick Children, London, UK
- Correspondence to:
Professor Dr Seza Ozen
Department of Paediatrics, Hacettepe University Faculty of Medicine, 06100 Ankara, Turkey; sezaozen{at}hacettepe.edu.tr
- Accepted 19 November 2005
- Published Online First 1 December 2005
Abstract
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
Footnotes
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Published Online First 1 December 2005
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↵* Under review by the ACR









