Refractory disease in antineutrophil cytoplasmic antibodies associated vasculitis.

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.