ANCA-associated vasculitis (AAV) is an autoimmune disease characterized by inflammation in small to medium-sized blood vessels, often involving the kidney. The activation of C5a in the alternative complement pathway mediates many important pro-inflammatory processes in the development of AAV. Avacopan, a selective antagonist of the C5a receptor, blocks C5a-mediated neutrophil activation and migration. These are two important steps in the destruction of the vascular endothelium.
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