Significant progress has been achieved in the management of ANCA-associated vasculitis and has resulted in enhanced patient outcomes since its initial characterization four decades ago. The administration of cyclophosphamide and/or B-cell depleting therapy in conjunction with glucocorticoids has been the fundamental approach in the treatment of severe conditions that pose a risk to organ function or life. However, recent clinical trials have undertaken a reassessment of established treatment approaches, while also exploring novel therapeutic targets. As a result, there has been a subsequent enhancement in the understanding and application of plasma exchange, the adoption of lower doses of oral glucocorticoids resulting in enhanced patient outcomes, and the exploration of additional therapy adjuncts or alternatives for minimizing steroid usage, such as C5a receptor antagonism. This chapter aims to analyze the advancements made in management of ANCA-associated vasculitis.