中文摘要：

目的：了解伴发急性/亚急性肺间质病变（interstitial lung disease,ILD）的临床无肌炎皮肌炎（clinical amyopathic dermatomyositis,CADM）患者的临床特点、不同治疗方案干预后随访2年的生存情况,并分析其危险因素。方法 ：回顾性分析上海交通大学医学院附属仁济医院风湿免疫科于2001年1月—2010年12月收治的伴发急性/亚急性ILD的CAMD患者101例,追踪2年的生存情况,采用Kaplan-Meier法比较不同治疗方案干预后2年的生存曲线,以COX回归分析该组患者的危险因素。结果：（1）CADM-ILD呈急进性ILD的临床模式,其2年存活率仅为43.5%,中位数存活时间为7个月;（2）随访CADM（101例）的病程为24个月,其中超过半数在1年内死亡,而病程〉12个月者预后较好;（3）Kaplan-Meier生存分析显示,联合激素＋免疫抑制剂方案（95%CI：14.98~18.58）优于单纯激素治疗方案（95%CI：3.41~13.59,P=0.001）;与硫唑嘌呤＋激素治疗方案（95%CI：8.49~12.61）、霉酚酸酯＋激素治疗方案（95%CI：12.24~19.52,P=0.351）相比,应用环孢素＋激素治疗方案的生存时间显著延长（95%CI：16.56~21.34,P〈0.001）;（4）通过COX回归分析,筛选到危险因素分别是早期发生低氧血症（RR=6.82,P=0.005）、血沉增快（RR=3.10,P=0.02）、铁蛋白增高（RR=6.27,P=0.003）及早期未联合使用免疫抑制剂（RR=2.73,P=0.001）。结论：CADM是炎症性肌病谱的组成部分,伴发ILD的CAMD呈急进性ILD的临床模式。在现有的各种药物治疗干预下伴发急性/亚急性ILD的CAMD预后仍不乐观,早期用药,激素联合免疫抑制剂治疗及加强支持治疗能延长患者的生存时间,其中环孢素的治疗方案较其他免疫抑制剂更佳。

英文摘要：

Objective： To investigate the two-year survival status of patients with clinically amyopathic dermatomyostitis complicated by acute/subacute interstitial disease（CAMD-A/SIP） receiving different treatment regimes, and to analyze its risk factors. Methods： 101 hospitalized patients with CAMD-A/SIP who received treatment during Jan. 2001 to Dec. 2010 in the Shanghai Renji Hospital were analyzed retrospectively. Kaplan-Meier analysis was used to compare the two-year-survival curve of patients receiving different treatment regimes. COX regression was used to analyze the risk factors of CAMD-A/SIP.Results：（1）CAMD-A/SIP showed a rapid progressive pattern. The two-year-survival rate of this group was only 43.5%, and median survival time was 7.0 months.（2）The follow-up time of our CAMD-A/SIP cohort（n=101） was 24 months. More than half of CAMD-A/SIP was refractory to conventional treatment.（3）Kaplan-Meier analysis showed that the two-year survival rate of patients receiving steroid alone（ 95 % CI ： 3. 41- 13. 59） was significantly lower than those receiving steroid ＋immunosupresants（95%CI： 14.98- 18.58, P=0.001）. Compared with azathiaprine ＋steroid（95%CI： 8.49- 12.61）, mycophenolate mofetil＋steroid（95%CI： 12.24- 19.52, P=0.351）, cyclosporine＋steroid achieved a significantly high suivival rate（95% CI：16.56- 21.34,P0.001）.（4）COX regression showed that regimes without immunosuppressants（RR=2.73,P=0.001）, acute type of interstitial disease（RR=6.27,P=0.003）, and lower pressure of oxygen at the beginning of treatment（RR=6.82,P=0.005） were risk factors for CAMD-A/SIP. Conclusion： CADM is constitutional of the disease spectrum, and CAMD-A/SIP is usually rapid progressive, which presents a special DM entity that deserves further study. The prognosis of CAMD-A/SIP is poor after treatment with existing regimes. Although the type of interstitial disease is one of the decisive factors early intervention,combined regimes including steroid and immunosuppres