The future of ANCA-associated vasculitis

The introduction of cyclophosphamide for treatment and the detection of antineutrophil cytoplasmatic antibodies (ANCA) as a seromarker for ANCA-associated vasculitis (AAV) have been the most important milestones in the history of AAV. Nevertheless, there are still many issues to resolve to fully understand the pathogenesis of AAV and to improve patient outcomes. There is a need for diagnostic criteria; treatment strategies need further improvement to reduce the toxicity of conventional immunosuppressants such as cyclophosphamide. The elucidation of the genetic background in patients with AAV and the role of granulomatous lesions found in Wegener's granulomatosis are required to fully understand the pathophysiology of AAV.