Department of Internal Medicine, Division of Nephrology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, Groningen 9713 GZ, The Netherlands.
Department of Internal Medicine, Division of Nephrology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, Groningen 9713 GZ, The Netherlands.
Autoimmun Rev. 2018 Apr;17(4):413-421. doi: 10.1016/j.autrev.2017.12.002. Epub 2018 Feb 9.
Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) are systemic autoimmune diseases characterized by necrotizing inflammation of small- to medium-sized blood vessels, affecting primarily the lungs and kidneys. Both animal and human studies show that the balance between inflammatory- and regulatory T- and B cells determines the AAV disease pathogenesis. Recent evidence shows malfunctioning of the regulatory lymphocyte compartment in AAV. In this review we summarize the immune regulatory properties of both T- and B cells in patients with AAV and discuss how aberrations herein might contribute to the disease pathogenesis.
抗中性粒细胞胞质抗体（ANCA）相关性血管炎（AAV）是一种系统性自身免疫性疾病，其特征为小至中等血管的坏死性炎症，主要影响肺和肾脏。动物和人类研究均表明，炎症性和调节性 T 细胞和 B 细胞之间的平衡决定了 AAV 的发病机制。最近的证据表明，AAV 患者的调节性淋巴细胞区室功能障碍。在这篇综述中，我们总结了 AAV 患者 T 细胞和 B 细胞的免疫调节特性，并讨论了这些异常如何导致疾病的发病机制。
