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Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) include Wegener granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and renal-limited vasculitis. This Review highlights the progress that has been made in our understanding of AAV pathogenesis and discusses new developments in the treatment of these diseases. Evidence from clinical studies, and both in vitro and in vivo experiments, supports a pathogenic role for ANCAs in the development of AAV; evidence is stronger for myeloperoxidase-ANCAs than for proteinase-3-ANCAs. Neutrophils, complement and effector T cells are also involved in AAV pathogenesis. With respect to treatment of AAV, glucocorticoids, cyclophosphamide and other conventional therapies are commonly used to induce remission in generalized disease. Pulse intravenous cyclophosphamide is equivalent in efficacy to oral cyclophosphamide but seems to be associated with less adverse effects. Nevertheless, alternatives to cyclophosphamide therapy have been investigated, such as the use of methotrexate as a less-toxic alternative to cyclophosphamide to induce remission in non-organ-threatening or non-life-threatening AAV. Furthermore, rituximab is equally as effective as cyclophosphamide for induction of remission in AAV and might become the standard of therapy in the near future. Controlled trials in which specific immune effector cells and molecules are being therapeutically targeted have been initiated or are currently being planned.