Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV), comprising microscopic polyangiitis, Wegener’s granulomatosis (now renamed as granulomatosis with polyangiitis), and Churg–Strauss syndrome, are life-threatening autoimmune inflammatory diseases commonly associated with renal failure. Previously almost universally fatal, the introduction of cyclophosphamide and corticosteroids revolutionized their treatment. The past 20 years has witnessed an ever-increasing interest in these diseases and a rapidly improving understanding of their complex pathogenesis. Recently, we have seen the publication of the first randomized controlled trials in AAV aimed at improving the treatment of the disease and minimizing treatment-associated adverse events. The advent of the era of biological therapies has seen the development of novel approaches to treating AAV based on improved insights into disease pathogenesis. This review will focus on the recent advances in our understanding of the pathogenesis of the disease, and evidence-based therapies. Int J Adv Rheumatol 2011;9(3):85–94.