The vasculitides associated with antineutrophil cytoplasmic antibodies (ANCAs) present to and are managed by a wide spectrum of physicians, reflecting the multi-organ nature of the conditions. Treatment strategies for these primary inflammatory vascular diseases have varied based on the outcomes of different clinical trials and practice reviews. The individual drugs used and their route of administration, dose, and duration of therapy have varied and have been the source of much debate. Advances in our understanding of disease immunopathogenesis, clinical assessment and outcome have formed the basis for several recent good-quality clinical trials. Now, with the results of these large-scale multicentre collaborative studies, there is a firmer evidence base to guide management decisions for individual patients. This evidence base, reviewed here, has led to the publication of treatment guidelines which importantly encompass many of the broader aspects of disease management.