Disorder of neutrophil extracellular traps in MPO-ANCA-associated vasculitis

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  • 好中球細胞外トラップの異常とMPO-ANCA 関連血管炎

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Abstract

MPO-ANCA-associated vasculitis (MPO-AAV) is necrotizing small vessel vasculitis usually involves renal glomeruli with production of MPO-ANCA. Although the pathogenic role of MPO-ANCA has been shown, the mechanism of its production remains unrevealed. A unique cell death of neutrophils, neutrophil extracellular traps (NETs), has recently been discovered. Although NETs are indispensable innate defense system, the disordered NETs could be related to autoimmune diseases, including MPO-AAV. Recently, we have demonstrated that the process of abnormal formation and impaired degradation of NETs induced by antithyroid drug propylthiouracil was involved in the generation of MPO-ANCA and subsequent development of MPO-AAV. On the other hand, IgG eluted from MPO-AAV sera demonstrated high ability for NETs induction, and the ability correlated to the disease activity and was parallel to the ANCA affinity to MPO. In addition, low ability of MPO-AAV serum for NETs degradation was determined. Correspondingly, activity of DNase I, an important physiological regulator of NETs, was generally low in MPO-AAV. Furthermore, the presence of anti-NETs antibodies, which could interfere with the DNase I activity, was demonstrated in some MPO-AAV sera. The collective findings suggest that “NETs-ANCA vicious cycle” could be critically involved in the pathogenesis of MPO-AAV.

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