摘要目的 分析不同肌炎特异性自身抗体(MSAs)对PM和DM患者预后的影响.方法 通过随访中日友好医院风湿科1994-2015年住院的PM/DM患者,分析不同MSA的PM/DM患者的Kaplan-Meier生存曲线,并用多因素Cox回归模型分析影响PM/DM患者预后的因素.结果 共383名PM/DM患者随访1～333个月,所有PM/DM患者累计生存率和10年生存率分别为68.6％和76.2％.其中MSAs阳性患者3年和5年的生存率为80.4％和77.1％,低于MSAs抗体阴性患者3年和5年的生存率(分别为90.1％和87.4％)(x2=3.90和3.98,P＜0.05).各MSAs组长期预后差异有统计学意义(x2=40.654,P＜0.01),其中抗黑色素分化相关基因5(MDA5)抗体阳性的PM/DM患者预后最差,10年生存率仅28.7％,而抗3-羟基-3-甲基-辅酶A还原酶蛋白(HMGCR)抗体阳性者预后最好,10年生存率为100％.多因素分析显示影响PM/DM患者生存的独立危险因素包括发病年龄、合并肿瘤、吞咽困难、合并快速进展性间质性肺病、抗MDA5抗体阳性、血清AST和CRP升高等.结论 不同MSAs对PM/DM患者的生存期有影响,MSA阳性者5年内的生存率更低,提示早期筛查MSAs并积极治疗可能改善患者的预后.

abstractsObjective To investigate the association of distinct myositis specific autoantibodies (MSAs) with long-term survival of patients with polymyositis (PM) and dermatomyositis (DM).Methods We analyzed the clinical data and outcome of patients with PM and DM who were hospita-lized in the department of rheumatology of China-Japan Friendship hospital from 1994 to 2015,and evaluated the impact of MSAs on the prognosis of patients.Multivariate Cox regression analysis was used to identify the prognostic risk factors for PM/DM patients.Results A total of 383 PM/DM patients were followed up for 1-333 months.Cumulative survival and 10-year survival rate of all patients were 68.6％ and 76.2％,respectively.The survival rate of 80.4％ and 77.1％ at 3 and 5 years in patients with MSAs,which were lower than those of patients with-out MSAs,who had the survival rate of 90.1％ and 87.4％ at 3 and 5 years,respectively(x2=3.90 and 3.98,P＜0.05).There was significant difference for long-term survival in all MSAs positive groups (x2=40.654,P=0.000).Anti-MDA5 positive patients who had the 10-year survival rate of 28.7％ had the worst prognosis,while anti-HMGCR positive patients who had the l0-year survival rate of 100％ had the best outcome in all groups.Multivariate Cox regression analysis showed that independent risk factors associated with the long-term survival of patients were age of onset,complicated with malignancies,dysphagia,rapidly progress interstitial lung disease,antiMDA5 antibody positive,increased serum aspartate transferase and C reaction protein.Conclusion MSAs are strongly associated with the prognosis of patients with PM/DM.Patients with MSAs has worse 5-year overall survival than those without MSAs,which indicates that screening MSAs and aggressive treatment for PM/DM patients at very early stage of disease may improve the outcome.