Disease activity assessment in childhood vasculitis: development and preliminary validation of the Paediatric Vasculitis Activity Score (PVAS).
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 de...
| Main Authors: | , , , , , , , , , , , , , , , , |
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| 格式: | Journal article |
| 语言: | English |
| 出版: |
2013
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| _version_ | 1826306975718178816 |
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| author | Dolezalova, P Price-Kuehne, F Özen, S Benseler, S Cabral, D Anton, J Brunner, J Cimaz, R O'Neil, K Wallace, C Wilkinson, N Eleftheriou, D Demirkaya, E Böhm, M Krol, P Luqmani, R Brogan, P |
| author_facet | Dolezalova, P Price-Kuehne, F Özen, S Benseler, S Cabral, D Anton, J Brunner, J Cimaz, R O'Neil, K Wallace, C Wilkinson, N Eleftheriou, D Demirkaya, E Böhm, M Krol, P Luqmani, R Brogan, P |
| author_sort | Dolezalova, P |
| collection | OXFORD |
| description | 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. |
| first_indexed | 2024-03-07T06:56:05Z |
| format | Journal article |
| id | oxford-uuid:fe2f0484-6d54-4dba-a3c4-0d8f2c7b0a45 |
| institution | University of Oxford |
| language | English |
| last_indexed | 2024-03-07T06:56:05Z |
| publishDate | 2013 |
| record_format | dspace |
| spelling | oxford-uuid:fe2f0484-6d54-4dba-a3c4-0d8f2c7b0a452022-03-27T13:34:27ZDisease activity assessment in childhood vasculitis: development and preliminary validation of the Paediatric Vasculitis Activity Score (PVAS).Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:fe2f0484-6d54-4dba-a3c4-0d8f2c7b0a45EnglishSymplectic Elements at Oxford2013Dolezalova, PPrice-Kuehne, FÖzen, SBenseler, SCabral, DAnton, JBrunner, JCimaz, RO'Neil, KWallace, CWilkinson, NEleftheriou, DDemirkaya, EBöhm, MKrol, PLuqmani, RBrogan, P 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. |
| spellingShingle | Dolezalova, P Price-Kuehne, F Özen, S Benseler, S Cabral, D Anton, J Brunner, J Cimaz, R O'Neil, K Wallace, C Wilkinson, N Eleftheriou, D Demirkaya, E Böhm, M Krol, P Luqmani, R Brogan, P Disease activity assessment in childhood vasculitis: development and preliminary validation of the Paediatric Vasculitis Activity Score (PVAS). |
| title | Disease activity assessment in childhood vasculitis: development and preliminary validation of the Paediatric Vasculitis Activity Score (PVAS). |
| title_full | Disease activity assessment in childhood vasculitis: development and preliminary validation of the Paediatric Vasculitis Activity Score (PVAS). |
| title_fullStr | Disease activity assessment in childhood vasculitis: development and preliminary validation of the Paediatric Vasculitis Activity Score (PVAS). |
| title_full_unstemmed | Disease activity assessment in childhood vasculitis: development and preliminary validation of the Paediatric Vasculitis Activity Score (PVAS). |
| title_short | Disease activity assessment in childhood vasculitis: development and preliminary validation of the Paediatric Vasculitis Activity Score (PVAS). |
| title_sort | disease activity assessment in childhood vasculitis development and preliminary validation of the paediatric vasculitis activity score pvas |
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