A patient with antineutrophil cytoplasm antibody-associated vasculitis frequently has multisystem disease and might present to a range of medical specialities. The manifestations that result in the greatest burden of morbidity and mortality are renal and pulmonary disease. In this review we will focus on rapidly progressive glomerulonephritis due to vasculitis, with specific reference to recent advances in our understanding of pathogenesis. The current standard of care for managing renal vasculitis, be it mild, moderate or severe, has largely been established in clinical trials of prednisolone, cyclophosphamide, methotrexate, azathioprine and plasma exchange. In addition to these, newer therapies such as mycophenolate mofetil and infliximab are being used more frequently. We will review the role of each of these approaches to management of small vessel vasculitis in 2005.