Emerging Therapies in Antineutrophil Cytoplasm Antibody-Associated Vasculitis

Shunsuke Furuta; David Jayne


Curr Opin Rheumatol. 2014;26(1):1-6. 

In This Article

Complement Targeted Therapies

Xing et al.[29] detected components of the alternative pathway, factor B, factor P, C3d and the membrane attack complex in glomeruli and small blood vessels in kidney biopsy specimens from AAV patients. This was supported by the absence of disease in an MPA model after factor B knockout or C5 knockout.[30] Moreover, Yuan et al.[31] reported that C5a was elevated in active AAV in both patients' plasma and urine. These studies suggested that complement activation via the alternative pathway was crucial for the disease development.

C5a is a cleavage product of complement C5 with strong chemotactic and anaphylatoxic features. CCX168 is a novel orally administered small molecule inhibitor of C5a receptor and a phase II trial (CLEAR) (ClinicalTrials.gov number; NCT01363388) is ongoing.