Table 1.

Teaching points

•Rapid control of AAV is paramount to prevent irreversible organ damage.

•Activation of the alternative complement pathway is a key step in propagating the inflammatory cascade in AAV.

•The anaphylatoxin C5a is a novel therapeutic target in AAV given its role in priming and recruiting neutrophils.

•New drugs targeting C5a have the potential to improve treatment efficacy and reduce treatment-related toxicity.

AAV, antineutrophil cytoplasmic antibody–associated vasculitis; C5a, complement component 5a.