Avacopan as First-Line Treatment in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Steroid-Sparing Option

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a rare autoimmune disease,S1 with high mortality in the absence of treatment.S2 Induction therapy with glucocorticoids (GC) plus either cyclophosphamide or anti-CD20 followed by 2 years of maintenance treatment reduces AAV-related mortality from 80% to ∼5%.S3 However, there is still an unmet need to define treatment strategies to reduce drug side effects including GC toxicity,S1 to reverse rare cases of refractory AAV, and to improve long-term outcomes.