This study reports on innovations in the field of, and current approaches to, the therapy of ANCA-associated vasculitis (AAV). Randomized clinical trials and prospective open label trial of newer therapies performed in the last 15 years in Wegener's granulomatosis and microscopic polyangiitis or both (AAV) were reviewed. Although cyclophosphamide remains the favoured immunosuppressive for remission induction, the use of alternative immunosuppressives and of intravenous pulsed administration have reduced cyclophosphamide exposure and are likely to increase the safety of treatment. Mycophenolate mofetil, leflunomide and deoxyspergualin are newer immunosuppressive drugs which have been evaluated in AAV, while tumpur necrosis factor, alemtuumab and rituximab are ‘biologic’ agents that have received attention. There is insufficient study of the dosing of glucocorticoids. Plasma exchange is indicated for severe renal vasculitis. Outcomes for elderly patients presenting with severe renal impairment are often poor despite optimal therapy. The development of collaborative networks in Europe and the USA has facilitated the conduct of larger randomized controlled trials. These have led to consensus recommendations on how AAV should be treated. Many newer agents are currently under evaluation which have the potential to improve AAV outcomes in the future.