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Disease activity assessment in childhood vasculitis: development and preliminary validation of the Paediatric
Pavla Dolezalova1, Fiona E Price-Kuehne, Seza Özen
1Rheumatology Unit, Department of Paediatrics and Adolescent Medicine, Charles University in Prague, 1st Faculty of Medicine and General University Hospital in Prague, Czech Republic. dolezalova.pavla@vfn.cz
Insights
A new Paediatric Vasculitis Activity Score (PVAS) was developed to reliably assess disease activity in children with rare chronic vasculitides. This validated tool aids clinical practice and therapeutic trials for better disease management.
Area of Science:
- Pediatric Rheumatology
- Clinical Trial Methodology
- Disease Assessment Tools
Background:
- Chronic childhood vasculitides require reliable disease activity assessment for effective management.
- Emerging treatments necessitate tools to quantify disease state changes in pediatric vasculitis.
Purpose of the Study:
- To develop and validate a modified Birmingham Vasculitis Activity Score (BVASv.3) for pediatric use.
- Establish a reliable Paediatric Vasculitis Activity Score (PVAS) for children.
Main Methods:
- Reviewed a pediatric vasculitis registry to identify missing clinical features in BVASv.3.
- Utilized a modified nominal group technique to create the PVAS.
- Conducted prospective validation for tool reliability, reproducibility, and responsiveness.
Main Results:
- The PVAS includes 64 items across nine categories, with 22 redefined BVAS items and 8 new pediatric-specific items.
- Demonstrated high interobserver agreement (linear-weighted-κ ≥0.87) for PVAS scores.
- PVAS showed significant correlation with physician assessment, treatment decisions, and ESR, and responded well to treatment.
Conclusions:
- The Paediatric Vasculitis Activity Score (PVAS) is a valid tool for assessing disease activity in children with systemic vasculitis.
- PVAS is anticipated to be a robust instrument for objectively defining disease activity in clinical trials and research.
Background:
Rare chronic childhood vasculitides lack a reliable disease activity assessment tool. With emerging new treatment modalities such a tool has become increasingly essential for both clinical practice and therapeutic trials to reproducibly quantify change in disease state.
Objective:
To develop and validate a paediatric vasculitis activity assessment tool based on modification of the Birmingham Vasculitis Activity Score (BVASv.3).
Methods:
A paediatric vasculitis registry was reviewed to identify clinical features missing in the BVASv.3. A modified nominal group technique was used to develop a working version of the Paediatric Vasculitis Activity Score (PVAS). Prospective validation provided tool reliability, reproducibility and responsiveness to change. Training of assessors was done according to the BVAS principles.
Results:
BVAS items were redefined (n=22) and eight paediatric items added in Cutaneous (n=4), Cardiovascular (n=3) and Abdominal (n=1) sections. The final PVAS has 64 active items in nine categories. The principles of new/worse and persistently active disease were retained as were the overall score and weighting of categories. The median PVAS in 63 children with systemic vasculitis was 4/63 (0-38/63). There was a high interobserver agreement for the overall as well as for subsystem scores (linear-weighted-κ ≥0.87). PVAS correlated with physician's global assessment (p<0.01); treatment decision (p=<0.01) and erythrocyte sedimentation rate (ESR) (p=0.01). In response to treatment, 15/19 patients assessed demonstrated a significant fall in PVAS (p=0.002), with good agreement among assessors for this change.
Conclusions:
The PVAS validity in children with systemic vasculitis was demonstrated. Like the BVAS, we anticipate that the PVAS will provide a robust tool to objectively define disease activity for clinical trials and future research.
