ANCA-associated Vasculitis: Focusing on Recent Advances in Medical Care

Abstract

ANCA-associated vasculitis is a necrotizing small vessel vasculitis associated with myeloperoxidase (MPO)- or proteinase 3 (PR3)-ANCA and is a systemic disease that affects various organs, including the kidney, lung, and nerves. New drugs and therapies are being investigated for both induction and maintenance of remission to improve efficacy and reduce side effects compared with conventional combination therapy with glucocorticoid and cyclophosphamide. Rituximab, an antibody preparation against CD20 antigen-positive B cells, has been shown to be non-inferior to cyclophosphamide for induction of remission and superior to azathioprine for maintenance of remission. In terms of side effects, rituximab is considered easier to use than cyclophosphamide. On the other hand, avacopan, a selective C5a receptor antagonist, has been shown to be an alternative to glucocorticoid during induction of remission and to have superior efficacy in preventing relapse compared to glucocorticoid during maintenance of remission. As for the glucocorticoid dosage, a randomized controlled trial (PEXIVAS) of plasma exchange therapy and glucocorticoid administration for ANCA-associated vasculitis showed no difference in remission rates and a low incidence of serious infections, even at about half the conventional dose of glucocorticoid.