Emerging therapies in antineutrophil cytoplasm antibody-associated vasculitis

Abstract

The current standard therapy for antineutrophil cytoplasm antibody-associated vasculitis (AAV), high-dose glucocorticoid and cyclophosphamide followed by azathioprine, has improved the disease prognosis. However, there are still unmet needs. For example, reducing relapse risk and glucocorticoid toxicity. Newer therapies are needed. Potential newer drugs are emerging following a better understanding of disease mechanisms and the availability of targeted therapies to B cells, T cells, proinflammatory cytokines and complement. Rituximab, an anti-CD20 monoclonal antibody, has proven efficacy in remission induction therapy for AAV, and two trials with rituximab as remission maintenance therapy are ongoing. Clinical trials evaluating mycophenolate mofetil as remission induction therapy, gusperimus, belimumab and complement factor C5a inhibition are also ongoing, and many other potential candidates are being investigated both clinically and experimentally. B-cell therapy is now an established treatment in AAV and several other therapies are under evaluation. However, the unmet need in vasculitis therapy remains large and newer therapies either alone or in combination will need to both improve efficacy and permit reductions in glucocorticoid and immunosuppressive exposure.