Patients with systemic vasculitis (SV), especially Wegener's granulomatosis and microscopic polyangütis, regularly present with renal involvement. Although considered a rare disease, either the incidence of SV is increasing or it is being increasingly recognized. Accurate classification systems are required to allow comparison of data from different groups investigating and treating these patients. Systemic vasculitis is known to be an autoimmune disease, but the mechanisms of pathogenesis have not been established, despite many studies on this topic in recent years. Most of this work has been done in vitro, although development of animal models is underway. Patient and renal survival have improved with aggressive immunosuppressive treatment, but morbidity is high and controversies remain in establishing the most effective regimens with minimum adverse effects. In this review we discuss the classification of SV, review the current knowledge of pathogenic mechanisms, and consider the relative merits of different treatment protocols.