Objectives: The knowledge of systemic necrotizing vasculitides improved since new classifications have been established along with a better understanding of pathogenesis of the diseases. The major vasculitides are described herein.
Current knowledge and key points: Pathogenesis plays now a major role for classifying diseases and influences the diagnostic strategies. The prospective therapeutic trials established by French and European groups are largely based on our better knowledge of the diseases.
Future prospects and projects: Despite the good results obtained with and demonstrated in prospective trials, the vasculitides remain severe and deserve new studies testing new drugs but also strategies based on prognostic factors and scores which should play a major role in treatments decision.