Purpose of review: Induction treatment of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) is not always successful and nonresponding patients are considered refractory.
Recent findings: Refractory disease should be subdefined to the treatment that was received. Cyclophosphamide refractory AAV occurs in up to 5% of patients. Many more patients develop contraindications to cyclophosphamide or relapse frequently. The latter two patient groups might also benefit from treatment used for cyclophosphamide refractory AAV.
Summary: The most promising drug for treating refractory AAV is rituximab.