New treatments of ANCA-associated vasculitis

The ANCA-associated systemic vasculides are, in many cases, life-threatening conditions. Combined treatment with corticosteroids and cyclophosphamide has improved their prognosis considerably. Five year survival does, however, still not exceed 75%, due to the occurrence of treatment resistant cases and the severe adverse effects of this treatment regimen. In this review new treatment modalities are discussed. In cases resistant to conventional therapy high dose intravenous immunoglobulin and T-cell directed biologicals, that is anti-thymocyte globulin (ATG) and humanised monoclonal antibodies to CD4 and CDw52, may be effective. Also, TNF-directed treatment is being used. Less severe cases may benefit from methotrexate treatment instead of cyclophosphamide. For maintenance of remission azathioprine has been proven to be as effective as cyclophosphamide so preventing the toxicity of long-term cyclophosphamide treatment. Relapses may also be prevented by treatment based on changes in ANCA-titers or by elimination of Staphylococcus aureus carriage which is associated with relapsing disease in Wegener's Granulomatosis. Several of those new treatment modalities are now being tested in controlled studies by the European Vasculitis Study Group (EUVAS).