血清IgA和SIgA抗PR3抗体对PR3 - ANCA相关血管炎器官受累和疾病活动性的影响

C. Sandin, P. Eriksson, M. Segelmark, T. Skogh, A. Kastbom
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引用次数: 14

摘要

循环免疫球蛋白(Ig)A类针对蛋白酶3 (PR3)的抗中性粒细胞细胞质抗体(ANCA)已经在ANCA相关的血管炎(AAV)中被报道。然而,分泌IgA (SIgA) PR3‐ANCA在以前没有报道过。在这项研究中,我们比较了血清SIgA PR3‐ANCA、IgA PR3‐ANCA和IgG PR3‐ANCA水平与疾病特征的关系。在诊断时患有AAV和PR3‐ANCA的73例患者中,在本研究取样时,84%的IgG PR3‐ANCA检测阳性,47%的IgA‐ANCA检测阳性,36%的SIgA PR3‐ANCA检测阳性。IgA和IgG PR3‐ANCA在采样时不同器官表现(即上呼吸道、肺或肾脏)的患者中表现相似。然而,SIgA PR3‐ANCA在累及上呼吸道的患者中较少出现。在活动性疾病期间,IgA PR3‐ANCA和SIgA PR3‐ANCA阳性患者的比例明显高于非活动性疾病。8例患者在活动性疾病发病后24个月内前瞻性取样。在这些患者中,与IgG PR3‐ANCA相比,IgA PR3‐ANCA和SIgA PR3‐ANCA在缓解诱导后更容易变为阴性。我们的研究结果表明,与IgG PR3‐ANCA相比,血清IgA PR3‐ANCA和SIgA PR3‐ANCA与AAV疾病活动性的关系更密切。需要进一步的研究来揭示这是否对疾病活动监测有影响。PR3‐ANCA同型的平均数量随着疾病活动性的增加而增加,表明在活动性疾病期间全球B细胞激活。
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IgA‐ and SIgA anti‐PR3 antibodies in serum versus organ involvement and disease activity in PR3‐ANCA‐associated vasculitis
Circulating immunoglobulin (Ig)A class anti‐neutrophil cytoplasm antibodies (ANCA) directed against proteinase 3 (PR3) have been reported in ANCA‐associated vasculitis (AAV) with mucosal involvement. However, secretory IgA (SIgA) PR3‐ANCA has not been reported previously. In this study we compared serum levels of SIgA PR3‐ANCA and IgA PR3‐ANCA with IgG PR3‐ANCA in relation to disease characteristics. Among 73 patients with AAV and PR3‐ANCA at diagnosis, 84% tested positive for IgG PR3‐ANCA, 47% for IgA‐ANCA and 36% for SIgA PR3‐ANCA at the time of sampling for the present study. IgA and IgG PR3‐ANCA were represented similarly among patients with different organ manifestations, i.e. upper airway, lung or kidney at time of sampling. However, SIgA PR3‐ANCA was significantly less represented among patients with upper airway involvement. During active disease, the proportions of IgA PR3‐ANCA and SIgA PR3‐ANCA‐positive patients were significantly higher compared to inactive disease. Eight patients were sampled prospectively during 24 months from onset of active disease. In these patients, IgA PR3‐ANCA and SIgA PR3‐ANCA turned negative more often after remission induction compared to IgG PR3‐ANCA. Our findings suggest that serum IgA PR3‐ANCA and SIgA PR3‐ANCA are related more closely to disease activity in AAV compared to IgG PR3‐ANCA. Further studies are required to reveal if this has implications for disease activity monitoring. The mean number of PR3‐ANCA isotypes increased along with disease activity, suggesting a global B cell activation during active disease.
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