Wüthrich R P
Klinik B für Innere Medizin, Kantonsspital St. Gallen.
Praxis (Bern 1994). 1994 Oct 25;83(43):1202-6.
Antineutrophil cytoplasmatic antibodies (ANCA) represent a heterogeneous group of autoantibodies targeting cytoplasmatic proteins in neutrophils and monocytes. They can be subdivided into c-ANCA which react with proteinase 3 (PR3) and p-ANCA which recognize myeloperoxidase (MPO). PR3 and MPO are found in the azurophilic granules of neutrophils. Activated neutrophils express these proteins on the cell surface; therefore, they can interact with ANCA. This interaction induces neutrophil degranulation with release of lytic enzymes and free oxygen-radicals. The vasculitic process is thus initiated. The c-ANCA can be detected mainly in Wegener's granulomatosis, and the titers can be used to monitor disease activity. The p-ANCA are mainly found in pauci-immune, rapidly progressive glomerulonephritides (RPGN). Recently, a host of additional inflammatory diseases have been associated with ANCA, for example ulcerative colitis and Crohn's disease. The pathogenic role of ANCA in these inflammatory bowel diseases is largely unknown.
抗中性粒细胞胞浆抗体(ANCA)是一类针对中性粒细胞和单核细胞胞浆蛋白的异质性自身抗体。它们可分为与蛋白酶3(PR3)反应的c-ANCA和识别髓过氧化物酶(MPO)的p-ANCA。PR3和MPO存在于中性粒细胞的嗜天青颗粒中。活化的中性粒细胞在细胞表面表达这些蛋白,因此它们可与ANCA相互作用。这种相互作用诱导中性粒细胞脱颗粒,释放溶解酶和游离氧自由基。血管炎过程由此启动。c-ANCA主要在韦格纳肉芽肿中检测到,其滴度可用于监测疾病活动。p-ANCA主要见于寡免疫性、快速进展性肾小球肾炎(RPGN)。最近,许多其他炎症性疾病也与ANCA相关,例如溃疡性结肠炎和克罗恩病。ANCA在这些炎症性肠病中的致病作用很大程度上尚不清楚。