Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitute a group of necrotizing systemic vasculitis with preferential involvement of small- to medium-sized vessels. None treated; they are considered as a life-threatening illness by their renal, cardiac and neurologic damages. Therefore, treatment is usually aggressive, with high-dose corticosteroid therapy combined with immunosuppressive drugs in the major part of cases. New biologic drugs have been introduced such as rituximab. In this chapter, we will present the update and recent advances in the treatment of AAV.