The clinical picture of primary systemic vasculitis, that cause inflammation and necrosis of vessel walls, depend on the type, size and location of involved vessels. Frequently, their clinical presentation does not reflect the specific type of vasculitis and its prognosis. Moreover, the correct diagnosis, early and adequate treatment have an important prognostic value. Recently, new diagnostic methods for systemic vasculitis have been devised. This article critically reviews and provides information for the rational use of these new imaging techniques and laboratory procedures for the diagnosis and follow up of systemic vasculitis.