Animal models for anti-neutrophil cytoplasmic antibody-associated vasculitis: Are current models good enough?

Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is a rare and severe systemic autoimmune disease characterized by pauci-immune necrotizing inflammation of small blood vessels. AAV involves multiple organ systems throughout the body. Our knowledge of the pathogenesis of AAV has increased considerably in recent years, involving cellular, molecular and genetic factors. Because of the controlled environment with no other confounding factors, animal models are beneficial for studying the mechanistic details of disease development and for providing novel therapeutic targets with fewer toxic side effects. However, the complexity and heterogeneity of AAV make it very difficult to establish a single animal model that can fully represent the entire clinical spectrum found in patients. The aim of this review is to overview the current status of animal models for AAV, outline the pros and cons of methods, and propose potential directions for future research.