Performance of Birmingham Vasculitis Activity Score and disease extent index in childhood vasculitides.

Objectives. 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. Methods. Patients fulfilling the EU-LAR/PRINTO/PRES (Ankara) c-vasculitis classification criteria for Henoch-Schonlein 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 1.5) for both tools. The performance characteristics of the BVAS and DEI with the unweighted methods were comparable. Conclusion. 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