Refractory vasculitis occurs in 4-5% of patients with anti-neutrophil cytoplasmic antibody associated vasculitis (AAV). Differences between therapies used for refractory disease are mostly reflected in the percentages of complete and partial remissions, but also in the number of serious side effects. Rituximab is considered the most safe second line therapy and should be advocated as a first alternative choice for cyclophosphamide in disease induction in refractory AAV.