Real-World Use of Avacopan in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Early and Long-Term Clinical, Renal, and Patient-Reported Outcomes in an Italian Rheumatology-Nephrology Network

Objective

Complement activation, particularly through the C5a-C5a receptor axis, plays a pivotal role in the pathogenesis of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) and may represent an important therapeutic target. Avacopan, an oral C5a receptor antagonist, was the first therapy targeting this pathway to enter in clinical practice. Prospective real-world data on its long-term effectiveness, safety, and impact on quality of life (QoL) remain limited. In the context of an evolving therapeutic and regulatory landscape, such data provide valuable evidence on the clinical effects of C5a receptor inhibition and may inform the development and evaluation of future complement-targeted therapies. The objective of this study was to evaluate the real-world effectiveness, safety, kidney outcomes, and patient-reported outcomes of avacopan in an Italian cohort of patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA).