The management of anti-neutrophil cytoplasmic antibody-associated vasculitis: what has changed in the last 10 years?

Abstract

The management of anti-neutrophil cytoplasmic antibody-associated vasculitis has substantially improved in the last decade. For the induction of remission, rituximab is increasingly used in place of cyclophosphamide, particularly for patients with proteinase 3 (PR3)-associated vasculitis or relapsing disease, and those wishing to preserve their fertility. A lower dose regimen of glucocorticoids, with a more rapidly tapering schedule, is preferable and is as effective and safer than the standard-dose regimen. Avacopan, the complement C5a receptor inhibitor, is effective in the treatment of associated vasculitis and may replace glucocorticoids in the future. Plasma exchange provides no additional benefit for patients with severe anti-neutrophil cytoplasmic antibody-associated vasculitis, although it is still used in selected patients on a case-by-case basis. Rituximab is preferred for the maintenance of remission, repeated at fixed time intervals. The duration for which immunosuppressive therapy should be given is uncertain, but is generally longer for patients with PR3 disease or persistent anti-neutrophil cytoplasmic antibody positivity. The anti-interleukin 5 monoclonal antibody, mepolizumab, is effective for the treatment of non-severe eosinophilic granulomatosis with polyangiitis. Several other targeted therapies are in the pipeline and further progress is expected in the coming years.