The introduction of glucocorticoids and cyclophosphamide has transformed ANCA associated vasculitis (AAV) from a fatal to a largely treatable condition. Over the past 40 years, considerable progress has been made in refining immunosuppressive regimens with a focus on minimising toxicity. As knowledge of the pathogenesis of AAV grows, it is mirrored by the availability of biological agents. Lymphocyte and cytokine targeted agents have been evaluated for the treatment of AAV and are entering the routine therapeutic arena with the potential to improve patient outcomes. Rituximab has transformed management of AAV in the past decade. However, there remains unmet need in the treatment of AAV; the majority of patients will relapse within five years of diagnosis despite maintenance immunosuppression; a small number of patients remain refractory to current therapies and treatment toxicity continues to contribute to mortality and chronic disability. As in rare diseases, treatment advances in vasculitis depend on international collaborative research networks to both establish an evidence base for newer agents and develop recommendations for optimal patient management.