Animal models of ANCA-associated vasculitides

Abstract

Antibodies against neutrophil proteins myeloperoxidase (MPO) and proteinase- 3 (PR3) are responsible for the development of anti-neutrophil cytoplasmic autoantibodies (ANCA)-associated vasculitides (AAV). Although the knowledge of these conditions is remarkably improved in the last few years, their etiology and pathogenetic mechanism(s) are still poorly understood. The establishment of experimental models has been repeatedly attempted with the aim of achieving a deeper understanding of their human counterpart. Here, we discuss the principal animal models currently used to investigate the mechanisms underlying the onset of AAV.