Savige J A, Davies D J
Department of Nephrology, Royal Melbourne Hospital, Parkville, Vic., Australia.
Aust N Z J Med. 1990 Jun;20(3):271-4. doi: 10.1111/j.1445-5994.1990.tb01038.x.
Circulating antibodies directed against cytoplasmic constituents of granulocytes and monocytes (anti-neutrophil cytoplasm antibodies, ANCA) are found in about 80% of active cases of Wegener's granulomatosis and microscopic polyarteritis. Antibodies are detected by indirect immunofluorescence on normal peripheral blood leucocytes, or by ELISA or radio-immunoassay using a neutrophil cytoplasm extract. There are 2 patterns of staining seen on indirect immunofluorescent examination of leucocytes and a number of different molecules recognised on Western blots. In Wegener's granulomatosis diffusely granular cytoplasmic staining (cANCA) is associated with a 29KD molecule that has recently been identified as neutrophil proteinase 3. A finely granular pattern may also be seen in microscopic polyarteritis. A perinuclear pattern (pANCA) is present in some other cases of segmental necrotising glomerulonephritis and occasionally in rheumatoid arthritis but almost never in Wegener's granulomatosis. This pattern is often associated with the presence of anti-myeloperoxidase and anti-elastase antibodies. Levels of anti-neutrophil cytoplasm antibodies usually reflect disease activity but a pathogenetic role for these antibodies in the development of small vessel vasculitides is, as yet, unproven.
在约80%的韦格纳肉芽肿病和显微镜下多动脉炎的活动病例中可发现针对粒细胞和单核细胞胞质成分的循环抗体（抗中性粒细胞胞质抗体，ANCA）。通过对正常外周血白细胞进行间接免疫荧光检测，或使用中性粒细胞胞质提取物通过酶联免疫吸附测定（ELISA）或放射免疫测定来检测抗体。在白细胞的间接免疫荧光检查中可看到两种染色模式，在蛋白质印迹法上可识别出许多不同的分子。在韦格纳肉芽肿病中，弥漫性颗粒状胞质染色（cANCA）与一种29KD的分子相关，该分子最近被鉴定为中性粒细胞蛋白酶3。在显微镜下多动脉炎中也可能出现细颗粒状模式。核周模式（pANCA）存在于一些其他节段性坏死性肾小球肾炎病例中，偶尔也见于类风湿关节炎，但在韦格纳肉芽肿病中几乎从不出现。这种模式通常与抗髓过氧化物酶和抗弹性蛋白酶抗体的存在有关。抗中性粒细胞胞质抗体的水平通常反映疾病活动，但这些抗体在小血管血管炎发展中的致病作用尚未得到证实。
