The vasculitides are a heterogeneous group of conditions typified by their ability to cause vessel inflammation with or without necrosis. They present with a wide variety of signs and symptoms and, if left untreated, carry a significant burden of mortality and morbidity. The ANCA-associated vasculitides (AAV) are three separate conditions - granulomatosis with polyangiitis (GPA - formerly known as Wegener’s granulomatosis); microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA - previously known as Churg-Strauss Syndrome). This review examines recent developments in the pathogenesis and treatment of AAV