Division of Nephrology, Department of Medicine, Hannover Medical School, Hannover, Germany.
Autoimmunity. 2009 Nov;42(7):605-14. doi: 10.1080/08916930903002503.
Much progress has been made in understanding the pathogenesis of anti-neutrophil cytoplasmic antibodies (ANCA)-associated small-vessel vasculitis and interaction between ANCA and micro-vascular endothelial cells are centre stage. The interactions of these key players culminate in respiratory burst of the neutrophil with release of radicals and proteases and subsequent endothelial cell and tissue damage. During the last decade, markers have become available to assess the extent and/or acuity of vascular damage in a clinical setting. First, circulating endothelial cells (CEC) have emerged as reliable surrogate markers of endothelial damage in vasculitis. More recently, endothelial microparticles have been used and appear to reflect damage and activation of the cells. Data on endothelial progenitor cells in vasculitis are sparse but intriguing while a genuine progenitor cell deficiency remains controversial. The severely damaged phenotype of CEC in vasculitis led to the hypothesis that such circulating apoptotic and/or necrotic debris may itself be a mediator of disease and first data from experimental studies have added proof to this assumption. Such effects may well contribute to a pro-inflammatory environment in ANCA-associated small-vessel vasculitis and in vascular disease in general. Here, we review mechanisms and markers of endothelial damage and repair in ANCA-associated vasculitis and put these findings into perspective.
在理解抗中性粒细胞胞浆抗体（ANCA）相关性小血管血管炎的发病机制方面已经取得了很大进展，ANCA 与微血管内皮细胞的相互作用成为研究的重点。这些关键因素的相互作用最终导致中性粒细胞呼吸爆发，释放自由基和蛋白酶，进而导致内皮细胞和组织损伤。在过去十年中，已经有一些标志物可用于评估血管损伤的程度和/或严重程度。首先，循环内皮细胞（CEC）已成为血管炎内皮损伤的可靠替代标志物。最近，内皮微颗粒已被用于反映细胞的损伤和激活。关于血管炎中内皮祖细胞的数据较少，但很有趣，而真正的祖细胞缺乏仍然存在争议。血管炎中 CEC 的严重损伤表型导致了这样的假设，即这种循环凋亡和/或坏死碎片本身可能是疾病的介质，来自实验研究的初步数据为此假设提供了证据。这些影响可能有助于 ANCA 相关性小血管血管炎和一般血管疾病中的促炎环境。在这里，我们综述了 ANCA 相关性血管炎中内皮损伤和修复的机制和标志物，并对这些发现进行了探讨。
