Immunotherapy for antineutrophil cytoplasmic antibody–associated vasculitis: challenging the therapeutic status quo?

Anti-neutrophil cytoplasmic antibodies (ANCA) are IgG antibodies directed against antigenic constituents of neutrophils contained in the azurophilic granules and monocyte lysosomes. Systemic vasculitis with ANCA [ANCA-associated vasculitides (AAV)] is a subgroup of life-threatening inflammatory disorders affecting small- to medium-sized vessels; immunosuppressants and glucocorticoids represent the current therapeutic mainstay. Although these agents have improved patients’ survival, 25% present with severe adverse events, and standard therapy does not sustain remission. Therefore, an unmet need for safer and more effective therapies has prompted interest in biological agents. Continuous advances in the knowledge of AAV pathogenesis are paving the way to new biologicals that are now awaiting testing.