【摘要】 目的 回顾性分析成人皮肌炎(DM)合并皮下钙化患者的治疗、生存率及预后的影响因素。方法收集于中日友好医院就诊的25例DM合并皮下钙化患者的临床资料并对其进行随访。采用t检验、Mann-Whitney U检验、Fisher精确检验、Kaplan-Meier生存分析、Log-rank检验及Cox回归进行统计分析影响皮下钙化患者预后的因素。结果25例患者中位存活时间为51个月,1、5、10年总生存率分别为95.8%、91.3%及91.3%,2例患者死因分别为淋巴瘤和感染;Kaplan-Meier分析结果显示,合并肿瘤以及肌炎特异性抗体(MSA)阴性的钙化患者死亡率高;钙化改善组较非改善组基线皮下钙化严重程度评分低(3分比4分,P=0.035)、V字征发生率高(100.0%比32.3%,P=0.033)和3个月内病情缓解率高(100.0%比41.2%,P=0.040)。进一步行Cox风险回归分析发现,治疗3个月病情缓解是皮下钙化改善的保护性因素(HR=0.236,P=0.040)。结论成人DM伴皮下钙化的患者总体死亡率低,但合并肿瘤及MSA阴性患者死亡率高;3个月病情缓解是皮下钙化改善的保护性因素,早期积极治疗,尽快控制疾病活动,有助于改善皮下钙化患者预后。更多还原

【Abstract】 Objective To analyze the treatment, survival rate and prognostic factors of adult dermatomyositis( DM) patients with calcinosis. Methods A retrospective analysis was performed on25 adult patients who admitted into China-Japan Friendship Hospital. T test,Mann-Whitney U test,Fisher test,Kaplan-Meier survival analysis and Log-rank test were used for statistical analysis. Results The medium survival time was 51 months. The survival rate at 1,5 and 10 years from the diagnosis were 95.8%,91.3%and 91.3% respectively. The causes of death in two patients were lymphoma and infection respectively.The mortality of patients associated with cancer and negative myositic specific antibody(MSA) was higher.Patients with calcinosis improvement had lower baseline calcification severity scores(3 points vs 4 points,P = 0. 035),higher rates of V sign( 100. 0% vs 32. 3%,P =0. 033) and remission in 3 months( 100. 0% vs41.2%,P = 0.040). Remission in 3 months was the protective factor of patients with calcinosis improvement(HR = 0. 236,P = 0. 040). Conculusion The mortality of adult DM patients with calciniosis is lower,but patients of calcinosis associated with cancer and negative MSA have higher mortality. Remission in3 months is the protective factor of patients with calcinosis improvement, which indicates that aggressive treatment for patients with calcinosis at very early stage of disease may improve the outcome.更多还原