影响多发性肌炎、皮肌炎患者预后的相关因素研究

A Study on the Related Factors Affecting the Prognosis of Patients with Polymyositis or Dermatomyositis

目的观察影响多发性肌炎(polymyositis,PM)和皮肌炎(dermatomyositis,DM)患者预后的相关因素,并探讨抗Jo1抗体在判断预后中的价值。方法对52例PM(27例)和DM(25例)患者进行1～27年随访,并对所有患者进行抗Jo1抗体测定,临床肌力评定应用4级功能障碍评级法,统计学处理应用科克斯比例危险模式、四格表确切概率法及χ2分析影响PM和DM患者预后相关因素。结果52例患者病死率21.2%,1年生存率86.2%,3年生存率81.4%,5年生存率78.4%,9年生存率74.6%,未发现与生存时间密切相关的因素。伴有恶性肿瘤(P=0.003)、间质性肺炎(P=0.006),吞咽困难(P=0.004)患者病死率高于不伴上述疾病患者,痊愈和基本治愈患者占生存患者的60.7%。发病时肌肉功能情况与预后无关。52例PM和DM患者抗Jo1抗体阳性9例(17.3%),其中PM阳性者6例(22.2%),DM阳性者3例(12.0%),抗Jo1抗体阳性患者66.7%伴间质性肺炎,33.3%伴吞咽困难。结论PM和DM患者长期预后较好,存活者中大部分患者可基本痊愈,伴有恶性肿瘤、间质性肺炎、吞咽困难患者病死率高。抗Jo1抗体阳性患者易伴间质性肺炎和吞咽困难。

Objective To assess the related factors including anti-Jo-1 antibody which affected the prognosis of polymyositis (PM) and dermatomyositis (DM). Methods 52 patients with PM and DM were followed up from 1 to 27 years, functional disability was assessed using a 4-stage grading system, anti-Jo-1 antibody was tested in all cases, statisticam methods included Cox proportional hazards model, Fisher’s exact test and χ 2 test computer analysis.Results Case-fatality rate was 21.2%, one-year survival rate 86.2%, three-year 81.4%, five-year 78.4%, nine-year 74.6%, the factor that closely related to survival time was not found. The case-fatality rate in the groups with malignant tumour (P=0.003), interstitial pneumonia (P=0.006), dysphagia (P=0.004) were higher than those without these diseases, the cured was 60.7% of survival patients. The musclar function at onset of the disease was not related to prognosis. Anti-Jo-1 antibody was positive in 6 of 27 PM (22.2%) and 3 of 25 DM (12.0%). In the group with positive anti-Jo-1 antibody, 66.7% patients accompanied with interstitial pneumonia, 33.3% dysphagia. Conclusions The long-term prognosis of PM and DM was better, the most survival patients could be almost cured,the prognosis of the patients with malignant tumour, interstitial pneumonia, and dysphagia was poor while case-fatality rate was high. The patients with positive anti-Jo-1 antibody were apt to be associated with interstitial pneumonia and dysphagia.