Refractory disease in antineutrophil cytoplasmic antibodies associated vasculitis

Purpose of reviewInduction treatment of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) is not always successful and nonresponding patients are considered refractory.Recent findingsRefractory 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.SummaryThe most promising drug for treating refractory AAV is rituximab.