摘要
抗中性粒细胞胞质抗体(anti-neutrophil cytoplasmicantibody,ANCA)相关性血管是一类严重的系统性自身免疫性疾病,特征表现为全身多系统寡免疫复合物沉积的小血管坏死性炎症和纤维素样坏死以及血清抗中性粒细胞胞质抗体阳性。目前被广泛认可的抗中性粒细胞胞质抗体相关性血管炎(ANCA associated vasculitis,AAV)发病机制包括ANCA介导的中性粒细胞活化、炎细胞因子和活性氧的释放以及中性粒细胞胞外陷阱的形成。现行AAV治疗方案仍为糖皮质激素联合免疫抑制剂,但该方案复发率高且不良反应较多。目前与免疫发病机制相关的最新研究发现免疫靶向治疗可以转化为临床实践。本文将总结目前对ANCA相关血管炎免疫发病机制的最新理解,并解释免疫靶点治疗将可能如何转化为临床实践,以期为基础研究及临床诊疗提供新思路。
Anti-neutrophil cytoplasmic antibody(ANCA)-associated vasculitis(AAV)is a group of severe systemic autoimmune diseases,characterized by systemic multi-organ necrotizing inflammation of small blood vessels with pauci-immune complex deposition,fibrinoid necrosis,and positive serum ANCA.The currently widely recognized pathogenic factors for AAV include ANCA-mediated neutrophil activation,release of inflammatory cytokines and reactive oxygen species(ROS),and formation of neutrophil extracellular traps(NETs).Glucocorticoids combined with immunosuppressants are currently available to the treatment of AAV,although potentially leading to a high recurrence rate and numerous adverse reactions.Recent studies related to immune pathogenesis have revealed that immunetargeted therapy can be translated into clinical practice.This article summarized the latest understanding of the immune pathogenesis of ANCA-associated vasculitis and explained how immune target therapy may be translated into clinical practice,aiming to provide new insights into basic research and clinical diagnosis and treatment.
作者
申萱
乔玉峰
Shen Xuan;Qiao Yu-feng(Department of General Practice,Shanxi Provincial People's Hospital,Affiliated to Shanxi Medical University,Taiyuan 031000,China;Department of Nephrology,Shanxi Provincial People's Hospital,Affiliated to Shanxi Medical University,Taiyuan 031000,China)
出处
《临床肾脏病杂志》
2025年第8期697-704,共8页
Journal of Clinical Nephrology
