Update on therapeutic approaches in ANCA-associated vasculitis

Standard-of-care therapy for antineutrophil cytoplasmic antibody-associated vasculitis (AAV) remains dependent on glucocorticoids (GCs) and immune suppression, especially rituximab, but patient outcomes are directly affected by only partial efficacy in controlling active disease, high levels of toxicity, and risks for subsequent relapse. GC dosing has been optimised in randomised controlled trials and the complement inhibitor, avacopan, introduced as an alternative to GCs. Improved understanding of the inflammatory and immune dysregulation in AAV has provided many cytokine, complement, and B cell targets, which are driving new drug development.