ANCA-associated vasculitis: from bench research to novel treatments

Small-vessel vasculitic syndromes such as Wegener granulomatosis and microscopic polyangiitis, which are associated with circulating antineutrophil cytoplasmic autoantibodies, are an important cause of renal failure. Current immunosuppressive regimens based on cyclophosphamide have significantly improved survival in patients with these conditions. However, such treatments are toxic, increase the risk of infection and do not cure the disease; fresh approaches are, therefore, required. An increased understanding of the pathogenesis of these syndromes has allowed the rational use of newer therapies such as rituximab, an anti-CD20 chimeric monoclonal antibody that depletes B cells. Further understanding of the disease pathogenesis is crucial to the development of novel targeted therapies, which are urgently required to improve patient prognosis. Future potential therapies include molecules that block signaling pathways that are essential in the pathogenesis of these diseases.