系统性硬化症患者的疾病相关自身抗体谱。
Disease-related autoantibody profile in patients with systemic sclerosis.
机构信息
a Department of Rheumatology and Clinical Immunology , Faculty of Medicine, School of Health Sciences, University of Thessaly , Larissa , Greece.
b Biomedical Section , Institute of Research and Technology Thessaly, Centre for Research and Technology Hellas (CERTH) , Larissa , Greece.
出版信息
Autoimmunity. 2017 Nov;50(7):414-421. doi: 10.1080/08916934.2017.1357699. Epub 2017 Jul 27.
BACKGROUND
Autoantibodies (autoAbs) help in diagnosis and predicting clinical phenotypes in systemic sclerosis (SSc).
AIM OF THE STUDY
To determine the clinical utility of 13 SSc-related autoAbs in SSc patients.
MATERIAL AND METHODS
A total of 131 consecutive patients with SSc (111 female, mean age 58.1 ± 14 years; 49 with diffused cutaneous SSc [dcSSc] and 82 with limited cutaneous SSc [lcSSc]) were analysed by a multiplex line immunoassay (Euroimmun) for autoantibodies (autoAbs) against 13 SSc-related antigens. A total of 22 patients with primary Raynaud phenomenon (RP), and 22 healthy controls were also analysed.
RESULTS
ANA by indirect immunofluorescence was present in 128 (97.7%) patients with SSc. Excluding anti-Ro52, 113 (89.3%) SSc patients were positive for at least one autoAb: anti-Topoisomerase I (anti-Topo) I abs in 54 (41.2%), anti-centromere proteins (anti-CENP) in 37 (28.2%, all reactive with centromere protein-A (CENPA) and centromere protein B (CENPB)), anti-RNA polymerase III(RP11) in 19 (14.5%), anti-RNA polymerase III(RP155) in 13 (9.9%), anti-fibrillarin in 4 (3.1%), anti-Ku in 6 (4.6%), anti-nucleolus-organizing region (anti-NOR90) in 8 (6.1%), anti-PM-Scl100 in 2 (1.5%), and anti-PM-Scl75 in 4 (3.1%). There was no immunoreactivity for Th/To or platelet-derived growth factor receptor (PDGFR). Overall, 102 (77.9%) SSc patients had autoAbs against Topo I, CENPA or CENPB, RP11 or RP155. Anti-Topo I abs were strongly associated with dcSSc, interstitial lung disease (ILD) (p < .001), pulmonary hypertension (PH) (p = .019) and ILD-PH (p = .003). Anti-CENPB abs were associated with lcSSc, and negatively associated with ILD. Anti-RP11 and anti-NOR90 abs were associated with male gender, and anti-NOR90 associated with ILD.
CONCLUSIONS
Anti-Topo I, anti-CENP, and anti-RNA pol III are the most prevalent autoAbs in SSc. Anti-Topo I and anti-NOR90 abs are associated with ILD and/or PAH.
背景
自身抗体（autoAbs）有助于系统性硬化症（SSc）的诊断和预测临床表型。
目的
确定 13 种与 SSc 相关的自身抗体在 SSc 患者中的临床应用价值。
材料和方法
通过 Euroimmun 的多重线免疫分析（LIA）对 131 例连续 SSc 患者（111 例女性，平均年龄 58.1±14 岁；49 例弥漫性皮肤 SSc [dcSSc]和 82 例局限性皮肤 SSc [lcSSc]）进行 13 种与 SSc 相关抗原的自身抗体（autoAbs）分析。还对 22 例原发性雷诺现象（RP）患者和 22 例健康对照者进行了分析。
结果
128 例（97.7%）SSc 患者间接免疫荧光法抗核抗体阳性。排除抗 Ro52 后，113 例（89.3%）SSc 患者至少有一种自身抗体阳性：54 例（41.2%）抗拓扑异构酶 I（抗 Topo）I 抗体，37 例（28.2%，均与着丝粒蛋白 A（CENPA）和着丝粒蛋白 B（CENPB）反应）抗着丝粒蛋白，19 例（14.5%）抗 RNA 聚合酶 III（RP11），13 例（9.9%）抗 RNA 聚合酶 III（RP155），4 例（3.1%）抗核仁组成区蛋白，6 例（4.6%）抗 Ku，8 例（6.1%）抗核仁组成区蛋白，2 例（1.5%）抗 PM-Scl100，4 例（3.1%）抗 PM-Scl75。没有 Th/To 或血小板衍生生长因子受体（PDGFR）的免疫反应。总的来说，102 例（77.9%）SSc 患者存在针对 Topo I、CENPA 或 CENPB、RP11 或 RP155 的自身抗体。抗 Topo I 抗体与 dcSSc、间质性肺病（ILD）（p<0.001）、肺动脉高压（PH）（p=0.019）和 ILD-PH（p=0.003）强烈相关。抗 CENPB 抗体与 lcSSc 相关，与 ILD 呈负相关。抗 RP11 和抗 NOR90 抗体与男性性别相关，抗 NOR90 与 ILD 相关。
结论
抗 Topo I、抗 CENP 和抗 RNA pol III 是 SSc 中最常见的自身抗体。抗 Topo I 和抗 NOR90 抗体与 ILD 和/或 PAH 相关。
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