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  • Oral presentation
  • Open Access

Preliminary validation of the paediatric vasculitis activity score (PVAS)

  • 1,
  • 1,
  • 2,
  • 3,
  • 4 and
  • 1
Pediatric Rheumatology20119 (Suppl 1) :O15

https://doi.org/10.1186/1546-0096-9-S1-O15

  • Published:

Keywords

  • Vasculitis
  • Polyarteritis Nodosa
  • Leukocytoclastic Vasculitis
  • Preliminary Validation
  • Street Hospital

Background

There is a paucity of evidence-based data for the treatment of primary systemic vasculitis (PSV) in childhood, partly due to the lack of a standardised outcome-measure for use in clinical trials. The Paediatric Vasculitis Activity Score (PVAS) is a quantitative clinical-index of manifestations of active disease, divided into 9 organ sub-systems.

Objective

To provide preliminary validation of the PVAS.

Methods

Children at Great Ormond Street Hospital NHS Trust with a diagnosis of PSV underwent simultaneous assessment of disease activity by 2 assessors. Scores were assessed for inter-observer variability and correlation with the physician’s global assessment of disease activity (PGA), ESR and CRP. Patients with newly diagnosed PSV were assessed twice: at diagnosis and 1 month, to assess tool-responsiveness to a change in disease state.

Results

23 children with PSV were studied - 48% male, 52% female. The diagnoses were: Behçet’s disease (n=7), Wegener’s granulomatosis (n=5), polyarteritis nodosa (n=5), cutaneous leukocytoclastic vasculitis (n=3), Cogan’s syndrome (n=1), microscopic polyangiitis (n=1) and unclassified vasculitis (n=1). Median PVAS was 1.5 (range 0-38). Bland-Altman analysis demonstrated high inter-observer agreement and Kappa analysis showed perfect agreement for 8/9 organ-system scores (K=1, p=0.00). Spearman’s rank showed correlation between PVAS and PGA (r s =0.87, 95%CI=0.71 to 0.94, p=0.00) and CRP (r s =0.54, 95%CI=0.10 to 0.81, p=0.02) but no correlation with ESR (r s = -0.1). Four newly diagnosed patients demonstrated a fall in the PVAS in response to therapy (change in median from 13.5/63 to 3.5/63), with good agreement for this change between assessors.

Conclusion

This study provides preliminary evidence that PVAS is a useful clinical measure of vasculitis disease-activity with good inter-observer reliability and correlates highly with the PGA and CRP.

Authors’ Affiliations

(1)
Dept of Rheumatology, Institute of Child Health and Great Ormond Street Hospital, London, UK
(2)
Hacettepe University Hospital, Ankara, Turkey
(3)
Alder Hey Children’s Hospital, and University of Liverpool, UK
(4)
Charles University 1st Medical Faculty, Prague, Czech Republic

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