EXAMINATION OF DISEASE SEVERITY IN SYSTEMIC VASCULITIS FROM THE NOVELPERSPECTIVE OF DAMAGE USING THE VASCULITIS DAMAGE INDEX (VDI)

Assessment of disease severity in systemic vasculitis encompasses mort ality, which is now uncommon, and morbidity, which is increasing in si gnificance. Morbidity includes permanent scars or damage, an evolving concept offering a novel perspective which may be particularly valuabl e in chronic disease. We have developed a method for assessing damage in systemic vasculitis, but the relationship between damage and diseas e severity was unknown. Therefore, we examined whether the number of i tems of damage or the pattern of damage varied with the severity of sy stemic vasculitis. We established the characteristics of severe diseas e by examining fatal vasculitis as an example of the most severe disea se possible. We then showed that more damage occurred in fatal vasculi tis, more systems were damaged, and critical damage akin to organ fail ure was more common in fatal than non-fatal vasculitis. These observat ions were reproduced in specific diagnostic groups, namely classical W egener's granulomatosis and systemic rheumatoid vasculitis. Thus, seve re disease was characterized by many items of damage, multisystem dama ge and critical damage. This pattern of damage was also seen in a subg roup of patients with non-fatal vasculitis, who also have severe disea se.