This issue of the Rheumatic Disease Clinics of North America provides the rheumatologist with the latest information on diagnosis and treatment of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Topics covered inlucde epidemiology, history, pathogenesis, autoantibodies in ANCA-associated vasculitis (including drug-induced ANCAs), pauci-immune renal disease, ocular manifestations, diagnostic approach (including mimickers), Wegener's Granulomatosis, Churg-Strauss Syndrome, microscopic polyangiitis, outcome measures, and the future of ANCA-Associated Vasculitis.