In vivo approaches to investigate ANCA-associated vasculitis: lessons and limitations.

Anti-neutrophil cytoplasm autoantibody (ANCA)-associated diseases are autoimmune conditions characterized by necrotizing inflammation of small blood vessels. The immunogenesis and etiology of these conditions are unknown, but our knowledge of the immunopathogenesis has increased considerably in recent years. In this review, we discuss the animal models currently used to investigate the mechanisms of vascular injury and to test novel therapies. We outline their advantages and limitations and propose potential directions for future research.