DEVELOPMENT AND INITIAL VALIDATION OF THE VASCULITIS DAMAGE INDEX FORTHE STANDARDIZED CLINICAL-ASSESSMENT OF DAMAGE IN THE SYSTEMIC VASCULITIDES

Objective. To develop and validate the Vasculitis Damage Index (VDI) f or the standardized clinical assessment of damage in the systemic vasc ulitides. Methods. Using a nominal group consensus approach, the Birmi ngham Vasculitis Group generated guiding principles for assessment of damage in all systemic vasculitides. Damage was defined as irreversibl e change resulting from scars, Consensus principles were developed int o the VDI, including guidelines for use, a list of items of damage, an d a glossary. Results. For 100 surviving patients with systemic vascul itis, the median VDI score at last observation was 3 (range 0-8), With in the Wegener's granulomatosis subgroup, the median VDI score for 12 non-survivors was higher than for 47 survivors (non-survivors median s core 7, interquartile range 5-8 versus survivors median score 4, inter quartile range 2-5; P = 0.003), VDI scores for 100 patients,vith syste mic vasculitis increased from initial presentation to last observation by a median score of 3 (range 1-4; P < 0.001), The VDI assesses more items and is more sensitive to change than other indices of damage (P < 0.001), Using the VDI, trained observers can produce moderately cons istent damage scores. Conclusion. The VDI is a sensitive, reproducible , comprehensive, and credible clinical tool for quantifying damage, Th e data presented herein should enable further validation and testing o f the VDI in specific vasculitic syndromes, and should facilitate the comparison of different therapies.