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Published on: March 6, 2019
EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides
S Ozen1, N Ruperto, M J Dillon
1Department of Paediatrics, Hacettepe University Faculty of Medicine, 06100 Ankara, Turkey, and Paediatric Nephrology, Institute of Child Health and Great Ormond Street Hospital for Sick Children, London, UK. sezaozen@hacettepe.edu.tr
Insights
New criteria classify childhood vasculitis based on vessel size, aiding diagnosis of conditions like Henoch-Schönlein purpura (HSP) and Kawasaki disease (KD). This aims for global acceptance in pediatric rheumatology.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Systemic Vasculitis
Background:
- Lack of standardized classification criteria for pediatric vasculitis.
- Need for specific diagnostic criteria for common childhood vasculitides.
Framework:
- General classification based on vessel size.
- Subcategorization of small vessel disease into granulomatous and non-granulomatous types.
- Development of specific criteria for Henoch-Schönlein purpura (HSP), Kawasaki disease (KD), childhood polyarteritis nodosa (PAN), Wegener's granulomatosis (WG), and Takayasu arteritis (TA).
Implementation:
- Two-phase project: Delphi technique and consensus conference.
- Involvement of international pediatric rheumatologists and nephrologists.
- Consensus defined as at least 80% agreement.
Implications:
- Provides a widely acceptable general classification for childhood vasculitides.
- Offers specific, pediatric-focused criteria for common vasculitic conditions.
- Aims to improve diagnosis and management of pediatric vasculitis globally.
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.
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