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.