The prevalence and clinical significance of anti-PUF60 antibodies in patients with idiopathic inflammatory myopathy.
The prevalence and clinical significance of anti-PUF60 antibodies in patients with idiopathic inflammatory myopathy.
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特发性炎症性肌病患者抗PUF60抗体的患病率及临床意义。
DOI：
10.1007/s10067-018-4031-4
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发表时间：
2018
影响因子：
3.4
通讯作者：
Peng Qing-Lin
中科院分区：
文献类型：
--
作者：
Zhang Ya-Mei;Yang Han-Bo;Shi Jing-Li;Chen He;Shu Xiao-Ming;Lu Xin;Wang Guo-Chun;Peng Qing-Lin
针对多u结合因子60kda蛋白（PUF60）的自身抗体在高加索皮肌炎（DM）患者中有报道。然而，它们在特发性炎性肌病（IIM）中的临床意义仍有待完全阐明。我们的目的是分析中国IIM患者中抗puf60抗体的患病率和临床意义。在我们的研究中，涉及388例IIM患者，301例疾病对照者和167例健康对照者（hc）。建立酶联免疫吸附试验（ELISA）检测血清抗puf60水平，并采用免疫印迹法验证。适当时采用非配对mann - whitneytest和Spearman相关分析。IIM患者中检测到抗puf60抗体的频率为10.6%（41/388）。亚组分析显示，抗puf60抗体在糖尿病中患病率为10%，在多发性肌炎（PM）中患病率为5.5%，在免疫介导的坏死性肌炎（IMNM）中患病率为10%，在肌炎重叠综合征中患病率为26.5%。在系统性红斑狼疮（SLE）、类风湿性关节炎（RA）和Sjögren综合征（SS）患者中也检测到puf60抗体，阳性率分别为17.3%、14.5和10.1%。有趣的是，抗puf60抗体经常在临床上无肌炎自身抗体的amyopathic dermatomyositis （CADM）患者和DM患者中被观察到。此外，具有抗puf60抗体的糖尿病患者皮肤溃疡的患病率更高。此外，对8名患有puf60抗体的糖尿病患者的纵向调查显示，抗体水平随着疾病缓解而下降。抗puf60抗体对肌炎没有特异性，因为在其他风湿病中也能检测到。进一步研究抗puf60抗体可能揭示全身性自身免疫性疾病的共同致病途径。
Autoantibodies against poly-U-binding factor 60 kDa protein (PUF60) have been reported in Caucasian dermatomyositis (DM) patients. However, their clinical significance in idiopathic inflammatory myopathy (IIM) remains to be fully clarified. Our objective was to analyze the prevalence and clinical significance of anti-PUF60 antibodies in a large cohort of Chinese IIM patients. In our study, 388 IIM patients, 301 disease controls, and 167 healthy controls (HCs) were involved. An enzyme-linked immunosorbent assay (ELISA) was developed to detect serum anti-PUF60 levels and was validated using immunoblotting methods. Unpaired Mann-WhitneyUtest and Spearman correlation analysis were used when appropriate. Anti-PUF60 antibodies were observed in IIM patients at a frequency of 10.6% (41/388). Subgrouping analysis revealed that the prevalence of anti-PUF60 antibodies was 10% in DM, 5.5% in polymyositis (PM), 10% in immune-mediated necrotizing myositis (IMNM), and 26.5% in myositis-overlap syndrome. Anti-PUF60 antibodies were also observed in systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and Sjögren’s syndrome (SS) patients at a positive rate of 17.3, 14.5, and 10.1% respectively. Intriguingly, anti-PUF60 antibodies were frequently observed in clinically amyopathic dermatomyositis (CADM) patients and DM patients without currently known myositis autoantibodies. Furthermore, DM patients with anti-PUF60 antibodies had higher prevalence of skin ulcerations. Moreover, longitudinal investigation in eight DM patients with anti-PUF60 antibodies revealed that the antibodies levels decreased with disease remission. Anti-PUF60 antibodies were nonspecific for myositis, since they could be detected in other rheumatic diseases. Further investigation of anti-PUF60 antibodies may reveal shared pathogenic pathways in systemic autoimmune disorders.