Prendecki Maria, Gurung Angila, Pisacano Noelle, Pusey Charles D
Centre for Inflammatory Disease, Department of Immunology and Inflammation, Imperial College London, Hammersmith Campus, Du Cane Road, London W12 0NN, United Kingdom.
Centre for Inflammatory Disease, Department of Immunology and Inflammation, Imperial College London, Hammersmith Campus, Du Cane Road, London W12 0NN, United Kingdom.
Immunol Lett. 2024 Dec;270:106933. doi: 10.1016/j.imlet.2024.106933. Epub 2024 Oct 1.
Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a group of rare systemic autoimmune diseases characterised by necrotising inflammation of small blood vessels and usually associated with circulating ANCA. The pathophysiology of AAV is complex, involving many aspects of the innate and adaptive immune system. Neutrophils are central to the pathogenesis of AAV as they are both the target of the autoantibody and effector cells mediating vascular injury. We describe mechanisms for ANCA induced activation of neutrophils, the pathogenic mechanisms by which this leads to endothelial cell injury, and how neutrophil crosstalk modulates other aspects of the immune system in AAV.
抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）是一组罕见的系统性自身免疫性疾病，其特征为小血管的坏死性炎症，通常与循环中的ANCA相关。AAV的病理生理学很复杂，涉及固有免疫系统和适应性免疫系统的多个方面。中性粒细胞在AAV的发病机制中至关重要，因为它们既是自身抗体的靶标，又是介导血管损伤的效应细胞。我们描述了ANCA诱导中性粒细胞活化的机制、由此导致内皮细胞损伤的致病机制，以及中性粒细胞间的相互作用如何调节AAV中免疫系统的其他方面。
