Pathogenic role of ANCA in small vessel inflammation and neutrophil function

Once thought as the mere epiphenomenon of a largely unknown inflammatory process, Anti-Neutrophil Cytoplasmic Antibodies (ANCA) have gained growing attention in recent years as key players in the pathogenesis of small vessel inflammation. Genetic studies as well as animal and in vitro models suggest that multiple factors induce the generation of ANCA, which in turn have the ability to promote neutrophil activation and progression towards neutrophil extracellular trap (NET) development. A vicious circle emerges as NETs endowed with ANCA targets promote ANCA generation. Besides consolidated pathogenic data, less is yet known about the precise role of ANCA in the clinical practice.