Vencovský J, Palecková A, Becvár R
Revmatologický ústav, Praha.
Cas Lek Cesk. 1992 Oct 9;131(19):577-81.
Antibodies against cytoplasm of neutrophil leucocytes (ANCA) with a high sensitivity are found in idiopathic necrotizing vasculitis and idiopathic glomerulonephritis with formation of sickles (IGS). C-ANCA are directed against the myeloid lysosomal enzyme--proteinase 3 and are found above all in Wegener's granulomatosis and microscopic polyarteritis. P-ANCA are directed mainly against myeloperoxidase, less against leucocytic elastase or lactoferrin and are found above all in microscopic polyarteritis, IGS, polyarteritis nodosa or Churg-Strauss syndrome. The elevated ANCA level correlates with the activity of the disease or precedes a relapse. These autoantibodies activate probably neutrophil leucocytes for respiratory exacerbation and degranulation which may lead to extensive tissue damage.
在特发性坏死性血管炎和伴有新月体形成的特发性肾小球肾炎(IGS)中发现对中性粒细胞胞浆具有高敏感性的抗体(ANCA)。C-ANCA 针对髓样溶酶体酶——蛋白酶 3,主要见于韦格纳肉芽肿病和显微镜下多血管炎。P-ANCA 主要针对髓过氧化物酶,较少针对白细胞弹性蛋白酶或乳铁蛋白,主要见于显微镜下多血管炎、IGS、结节性多动脉炎或变应性肉芽肿性血管炎。ANCA 水平升高与疾病活动度相关或先于复发。这些自身抗体可能激活中性粒细胞以加剧呼吸爆发和脱颗粒,这可能导致广泛的组织损伤。