Activity, damage and outcome in systemic vasculitis

Current therapy has transformed the prognosis of the systemic vasculitides from diseases that had a high acute mortality rate to chronic relapsing diseases with high rates of morbidity. This improved survival has highlighted the need for better methods of monitoring disease activity and recording the accumulation of organ damage that occurs during the course of the disease. Several clinical indices have been developed that record disease activity, damage and the extent of disease. These validated indices allow a detailed assessment of the patient's response to therapy and provide an essential tool for insuring uniformity of patient monitoring in multi-centre trials. In addition, more data are now available supporting the use of these assessment tools as prognostic and outcome criteria for clinical studies. The development, properties, application and inter-relationships of the available clinical assessment tools for patients with systemic vasculitis are reviewed in this chapter.