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To evaluate the performance of the Birmingham Vasculitis Activity Score (BVAS) v3 and the Disease Extent Index (DEI) for the assessment of disease activity in 4 primary childhood (c-) systemic vasculitides.Patients fulfilling the EULAR/PRINTO/PRES (Ankara) c-vasculitis classification criteria for Henoch-Schönlein purpura (HSP), childhood (c) polyarteritis nodosa (c-PAN), c-Wegener's granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) with disease duration at the time of diagnosis ≤3 months were extracted from the PRINTO database. The performance of the BVAS and DEI were examined by assessing convergent validity, the pattern of disease involvement, and responsiveness. We also evaluated alternative unweighted scoring methods for both tools.The analysis set included 796 patients with 669 HSP, 80 c-PAN, 25 c-WG and 22 c-TA. The median age at diagnosis was 6.9 years (6.6-12) and median delay in making the diagnosis from the onset of signs/symptoms was 0.01 (0.003-0.027) years. A strong correlation was found between the BVAS and DEI (rs=0.78) while correlation with the physician global assessment was moderate (rs=0.48) with BVAS and poor with DEI (rs=0.25). Both the BVAS and DEI sub-scores and total scores were able to descrive the disease involvement in the 4 childhood vasculitides. Responsiveness was large (>1.5) for both tools. The performance characteristics of the BVAS and DEI with the unweighted methods were comparable.This study demonstrates that both the BVAS and DEI are valid tools for the assessment of the level of disease activity in a large cohort of childhood acute and chronic vasculitides.

Type

Journal article

Journal

Clinical and experimental rheumatology

Publication Date

01/2012

Volume

30

Pages

S162 - S168

Addresses

IRCCS G. Gaslini, Pediatria II, Reumatologia, PRINTO, Genova, Italy. dottore_erkan@yahoo.com

Keywords

Paediatric Rheumatology International Trials Organization (PRINTO), Humans, Polyarteritis Nodosa, Purpura, Schoenlein-Henoch, Vasculitis, Diagnosis, Differential, Prognosis, Health Status Indicators, Severity of Illness Index, Reproducibility of Results, Predictive Value of Tests, Child, Terminology as Topic, Takayasu Arteritis, Granulomatosis with Polyangiitis