Csernok Elena
Department of Rheumatology, University of Lübeck, Oskar-Alexander-Str. 26, 24576 Bad Bramstedt, Germany.
Autoimmun Rev. 2003 May;2(3):158-64. doi: 10.1016/s1568-9972(03)00010-7.
Anti-neutrophil cytoplasmic antibodies (ANCA) are a heterogenous group of autoantibodies with a broad spectrum of clinically associated diseases. ANCA testing has been established as a useful tool for the diagnosis of small vessel vasculitides, especially of 'ANCA-associated vasculitides' (AAV), such as Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome, in which circulating ANCA are commonly found. Within the last 20 years these antibodies were subject of intensive studies and a growing body of evidence arose for a distinct role of ANCA in the pathogenesis of the AAV. Our current concept of whether ANCA directly or indirectly contribute to vascular damage (ANCA-cytokine-sequence-theory) was mainly developed from in vitro studies and is supported by data from clinical investigations as well as animal models. Recently a direct causal link between ANCA and the development of glomerulonephritis and vasculitis has been demonstrated. We now know that a passive transfer of ANCA is sufficient to induce disease, but it remains to be discovered how the autoantibodies to neutrophil antigens might triggered.
抗中性粒细胞胞浆抗体（ANCA）是一类异质性自身抗体，与多种临床相关疾病有关。ANCA检测已成为诊断小血管炎的有用工具，特别是“ANCA相关血管炎”（AAV），如韦格纳肉芽肿、显微镜下多血管炎和变应性肉芽肿性血管炎，这些疾病中通常可检测到循环ANCA。在过去20年里，这些抗体一直是深入研究的对象，越来越多的证据表明ANCA在AAV发病机制中具有独特作用。我们目前关于ANCA是直接还是间接导致血管损伤的概念（ANCA-细胞因子序列理论）主要基于体外研究得出，并得到了临床研究和动物模型数据的支持。最近，已证实ANCA与肾小球肾炎和血管炎的发生之间存在直接因果关系。我们现在知道，被动转移ANCA足以诱发疾病，但中性粒细胞抗原自身抗体是如何触发的仍有待发现。
