Papo T, Lê Thi Huong D, Piette J C, Godeau P
Service de médecine interne, CHU Pitié-Salpétrière, Paris, France.
Rev Med Interne. 1994 Feb;15(2):110-5. doi: 10.1016/s0248-8663(05)81183-x.
ANCA mainly recognize proteinase 3 (cytoplasmic fluorescence) in Wegener's granulomatosis and myeloperoxidase (perinuclear fluorescence) in other types of vasculitis. A causative role of ANCA seems not obvious because: a) active generalized Wegener's granulomatosis may exist with no detectable ANCA, b) ANCA titres don't reliably parallel clinical activity and c) of the increasing diversity of clinical situations where ANCA are described. Nevertheless, some lines of evidence point to a pathogenetic role of ANCA, especially in vascular lesions genesis: a) ANCA may prevent inactivation of antigenic proteases by antiproteases, b) translocation at the outer membrane of intracellular antigens make them accessible to ANCA, c) activation of polymorphonuclear leucocytes and increase of their aggregation and cytotoxicity to endothelial cells are specifically induced by ANCA, d) proteinase 3 and myeloperoxidase may adhere to the endothelial cell membrane and then be recognized by ANCA, e) a specific T-lymphocyte response to ANCA antigens could be elicited in Wegener's granulomatosis and f) an in vivo model of ANCA-related glomerulonephritis has recently been established.
抗中性粒细胞胞浆抗体（ANCA）主要识别韦格纳肉芽肿中的蛋白酶3（胞质荧光）以及其他类型血管炎中的髓过氧化物酶（核周荧光）。ANCA的致病作用似乎并不明显，原因如下：a）活动期全身性韦格纳肉芽肿可能存在而检测不到ANCA；b）ANCA滴度与临床活动度并无可靠的平行关系；c）ANCA被描述的临床情况越来越多样化。然而，一些证据表明ANCA具有致病作用，尤其是在血管病变的发生过程中：a）ANCA可能会阻止抗蛋白酶使抗原性蛋白酶失活；b）细胞内抗原在外膜的易位使其可被ANCA识别；c）ANCA可特异性诱导多形核白细胞的活化及其聚集，并增强其对内皮细胞的细胞毒性；d）蛋白酶3和髓过氧化物酶可能会黏附在内皮细胞膜上，然后被ANCA识别；e）在韦格纳肉芽肿中可引发针对ANCA抗原的特异性T淋巴细胞反应；f）最近建立了ANCA相关性肾小球肾炎的体内模型。
