Yoshida Masaharu
Department of Nephrology, Hachioji Medical Center of Tokyo Medical University, Hachioji 193-0998.
Rinsho Byori. 2003 Jul;51(7):644-8.
ANCA are associated with small sized vessel vasculitis; one subtype is an antibody against myeloperoxidase(MPO), which stains in a perinuclear pattern(P-ANCA) indirect immunofluorescence(IIF) using a neutrophil substrate, and the other subtype is an antibody against proteinase-3(PR-3), which stains in a diffuse granular cytoplasmic pattern ANCA by IIF. PR-3 ANCA is more specific in Wegener's granulomatosis(WG) than the other primary vasculitides. MPO-ANCA can be found in microscopic polyangiitis (MPA), Churg Strauss Syndromes(CSS), drug-induced vasculitis, and environmental factor-induced such as silicosis vasculitis more frequently than WG. The value of the IIF test for ANCA detection can be greatly increased by the addition of a standardized antigen-specific ELISA. The intra-assay and inter-assay CV of the MPO and PR-3 ELISA were 6.6 to 4.8%, respectively. Close ANCA titer correlation was shown between MPO-ANCA ELISA and the activity of ANCA associated vasculitis. Renal manifestations and pulmonary manifestations are observed in 70-90% of AAV as the initial manifestation. The changes in titers of ANCA seem to reflect disease activity in 60-70% of AAV patients. A combination of steroids and immunosuppressive drugs is effective in relieving the clinical symptoms of AAV.
抗中性粒细胞胞浆抗体(ANCA)与小血管炎相关;其中一个亚型是抗髓过氧化物酶(MPO)抗体,在使用中性粒细胞底物的间接免疫荧光法(IIF)中呈核周型(P-ANCA)染色,另一个亚型是抗蛋白酶-3(PR-3)抗体,在IIF中呈弥漫性颗粒状胞浆型ANCA染色。PR-3 ANCA在韦格纳肉芽肿(WG)中比其他原发性血管炎更具特异性。与WG相比,MPO-ANCA在显微镜下多血管炎(MPA)、变应性肉芽肿性血管炎(CSS)、药物性血管炎以及环境因素诱导的血管炎(如矽肺血管炎)中更常见。通过添加标准化的抗原特异性酶联免疫吸附测定(ELISA),可大大提高IIF检测ANCA的价值。MPO和PR-3 ELISA的批内和批间变异系数分别为6.6%至4.8%。MPO-ANCA ELISA与ANCA相关血管炎的活性之间显示出密切的ANCA滴度相关性。70%至90%的抗中性粒细胞胞浆抗体相关性血管炎(AAV)患者最初表现为肾脏和肺部表现。在60%至70%的AAV患者中,ANCA滴度的变化似乎反映了疾病活动。类固醇和免疫抑制药物联合使用可有效缓解AAV的临床症状。