Division of Nephrology, Department of Medicine, Heersink School of Medicine, University of Alabama at Birmingham, Birmingham, Alabama.
Kidney360. 2023 Dec 1;4(12):1794-1805. doi: 10.34067/KID.0000000000000289. Epub 2023 Nov 6.
ANCA-associated vasculitis (AAV) belongs to a group of small vessel systemic vasculitides characterized by granulomatous and neutrophilic inflammation of various tissues. Patients often have circulating autoantibodies targeting neutrophilic antigens. Although AAV was once associated with severe end-organ damage and extremely high mortality rates, the use of glucocorticoids and cyclophosphamide led to a paradigm change in its treatment. Over the past 20 years, significant progress in understanding the immunopathogenesis of AAV has enabled development of targeted immunotherapies, providing a much better prognosis for patients. This review describes the evolution of treatment of AAV, particularly for patients with kidney involvement.
抗中性粒细胞胞浆抗体相关性血管炎（AAV）属于一组小血管系统性血管炎，其特征是各种组织的肉芽肿性和中性粒细胞炎症。患者通常有针对中性粒细胞抗原的循环自身抗体。尽管 AAV 曾与严重的终末器官损伤和极高的死亡率相关，但糖皮质激素和环磷酰胺的使用导致了其治疗模式的转变。在过去的 20 年中，对 AAV 的免疫发病机制的理解取得了重大进展，从而开发了靶向免疫疗法，为患者提供了更好的预后。本文综述了 AAV 的治疗进展，特别是针对肾脏受累患者的治疗进展。
