Bosch X, Mirapeix E, Font J, López-Soto A, Rodríguez R, Vivancos J, Revert L, Ingelmo M, Urbano-Márquez A
Servicio de Medicina Interna General, Hospital Clínic i Provincial, Universidad de Barcelona.
Med Clin (Barc). 1994 Mar 26;102(11):412-7.
The prevalence and diagnostic usefulness of antineutrophil cytoplasmic antibodies (ANCA) were analyzed in a series of patients with different types of vasculitis, connective tissue diseases, nephropathies and lung diseases.
Six hundred seventy-six consecutive patients with systemic vasculitis, connective tissue diseases, nephropathies and different lung disturbances were included in the study. The ANCA were determined by indirect immunofluorescence (IIF) on neutrophils fixed in ethanol. For the detection of antimyeloperoxidase (MPO) antibodies (Ab) a new technique was developed consisting in the performance of IIF on neutrophils with a deficiency in MPO. The serologic samples were also analyzed by enzyme-linked immunosorbent assays with MPO and purified proteinase 3 (PR3).
ANCA were demonstrated in 95 patients. A cytoplasmic pattern (c-ANCA) was detected in 25 cases (26%) and in 70 (74%) a perinuclear pattern (p-ANCA) was observed. Twenty-one of the 25 cases (84%) of c-ANCA corresponded to anti-PR3 Ab and 61 of the 70 (87%) of p-ANCA corresponded to anti-MPO Ab. The anti-PR3 Ab identified Wegener's granulomatosis, with a sensitivity and specificity for the generalized and clinically active forms being 65 and 88%, respectively. The anti-MPO Ab were principally detected in patients with rapidly progressive glomerulonephritis (RPGN) and hemorrhagic alveolar capillaritis. The sensitivity and specificity of the anti-MPO Ab for the RPGN and alveolar capillaritis were 75% and 98%.
Two principal subtypes of ANCA may be detected, which also recognize two fundamental clinicopathologic entities. The high diagnostic sensitivity and specificity of these antibodies for each of the processes to which they are associated can justify the adoption of therapeutic measures in determined cases.
分析了一系列不同类型血管炎、结缔组织病、肾病和肺病患者中抗中性粒细胞胞浆抗体(ANCA)的患病率及其诊断价值。
本研究纳入了676例连续性的系统性血管炎、结缔组织病、肾病及不同肺部疾病患者。采用乙醇固定的中性粒细胞间接免疫荧光法(IIF)检测ANCA。为检测抗髓过氧化物酶(MPO)抗体,开发了一种新技术,即对MPO缺陷的中性粒细胞进行IIF检测。血清样本还采用MPO和纯化蛋白酶3(PR3)的酶联免疫吸附试验进行分析。
95例患者检测到ANCA。25例(26%)检测到胞浆型(c-ANCA),70例(74%)观察到核周型(p-ANCA)。25例c-ANCA中有21例(84%)对应抗PR3抗体,70例p-ANCA中有61例(87%)对应抗MPO抗体。抗PR3抗体可识别韦格纳肉芽肿,对全身性和临床活动型的敏感性和特异性分别为65%和88%。抗MPO抗体主要在快速进展性肾小球肾炎(RPGN)和出血性肺泡毛细血管炎患者中检测到。抗MPO抗体对RPGN和肺泡毛细血管炎的敏感性和特异性分别为75%和98%。
可检测到两种主要的ANCA亚型,它们也识别两种基本的临床病理实体。这些抗体对与其相关的每个疾病过程具有较高的诊断敏感性和特异性,这可以为特定病例采取治疗措施提供依据。