High-dose corticosteroids in combination with cytotoxic drugs are universally accepted as the initial treatment in ANCA-associated vasculitis. Cyclophosphamide is the most effective cytotoxic drug and is used in more severe cases. Because of short- and long-term side-effects, novel therapies have been tested. New prospects for the treatment of vasculitis include sequential chemotherapy (e.g. cyclophosphamide followed by azathioprine or cyclophosphamide followed by methotrexate) and/or novel immunosuppressive agents (e.g. mycophenolate mofetil, anti-TNF-alpha).