中文摘要：

目的 检测抗PUF60抗体在中国特发性炎性肌病（idiopathic inflammatory myopathy,IIM）患者中的阳性率,并探索其临床意义。方法 选取1989年10月至2016年8月于中日友好医院住院治疗的388例IIM患者[275例皮肌炎（dermatomyositis,DM）、76例多发性肌炎（polymyositis,PM）及37例肌炎重叠综合征],211例其他自身免疫性疾病患者[28例神经肌肉疾病（noninflammatory neuromuscular disease,NMD）、104例系统性红斑狼疮（systemic lupus erythematosus,SLE）及79例干燥综合征（Sj9gren syndrome,SS）]作为疾病对照组;同时选取门诊体检的167例健康成年人作为健康对照组（healthy controls,HCs）。采用酶联免疫吸附法（enzyme-linked immunosorbent assay,ELISA）检测入组患者及HCs的抗体滴度,并联合免疫印迹实验验证ELISA结果的可靠性。组间比较用独立Mann-Whitney U检验,相关性分析采用Spearman秩检验。结果 抗PUF60抗体可见于11.6%（45/388）的IIM患者,17.3%（18/104）的SLE患者,10.1%（8/79）的SS患者,阳性率均高于HCs（IIM比HCs,P〈0.001;SLE比HCs,P〈0.001;SS比HCs,P=0.009）。且该抗体可见于各IIM亚组,包括11.3%（31/275）的DM患者和21.6%（8/37）的肌炎重叠综合征患者,阳性率均高于HCs（DM比HCs,P〈0.001;肌炎重叠综合征比HCs,P〈0.001）。此外,抗PUF60抗体在不同自身免疫性疾病中的临床相关性各异。在经典DM中,该抗体常见于肌炎抗体阴性（P=0.018）及并发技工手（P=0.046）的患者;而且该抗体在无肌病性皮肌炎中的阳性率较高,且多伴发皮肤溃疡（P=0.029）。在其他自身免疫性疾病中发现,抗体阳性的SS患者皮疹发生率及类风湿因子、抗SSB抗体、抗RNP抗体阳性比例增加,且抗体阳性的SLE患者外周血CD5＋CD19＋B细胞数目升高及抗双链DNA、抗Sm、抗SSB抗体阳性比例同样增加（P均〈0.05）。结论 抗PUF60抗体可见于中国DM、肌炎重叠综合征、SLE和SS患者,且在不同类型自身?

英文摘要：

Objective The aim of our study was to detect the positive incidence and analyze the clinical significance of anti- PUF60 antibody in Chinese patients with idiopathic inflammatory myopathy （IIM ）. Methods This study involved, from October 1989 to August 2016, 388 IIM patients （including 275 patients with dermato- myositis （DM） , 76 patients with polymyositis （PM） , and 37 patients with myositis overlap syndrome） , 211 pa-tients with control diseases （including 28 patients with noninflammatory neuromuscular disease （ NMD） , 104 pa-tients with systemic lupus erythematosus （ SLE） , and 79 patients with Sjogren’s syndrome （SS） ） as disease controls, and 167 healthy adults as healthy controls （HCs）. Enzyme-linked immunosorbent assay （ELISA） was developed to detect serum anti- PUF60 antibody, and immunoblotting analyses were performed to validate the ELISA results. The results of two groups were compared using unpaired Mann-Whitney U test, and the relevance was analyzed using Spearman correlation analysis. Results Anti- PUF60 antibody was detected in 11. 6% （45/388） of patients with I IM , 17. 3% （18/104） of patients with SLE, and 10. 1% （8/79） of patients with SS, and the antibody positive rates were significantly higher than that of HCs （P〈0.001, P〈0.001, and P = 0.009, respectively）. In IIM subgroups, anti- PUF60 antibody presented in 11.3% （31/275） of DM patients and 21. 6% （8/37） of patients with myositis overlap syndrome, and the antibody positive rates were significantly higher than that of HCs （P =0.001 and P 〈0.001, respectively）. The clinical associations of anti- PUF60 anti-body in different groups of immune disease were varied. Classical DM patients with anti- PUF60 antibody had lower prevalence of currently known myositis antibodies （ P = 0. 018） and higher prevalence of mechanic’s hands （P = 0. 046）. While, the prevalence of anti- PUF60 antibody was particularly hig