临床无肌炎皮肌炎伴急性/亚急性肺间质病变101例回顾性分析

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。%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,P<0.001). (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 immunosuppresants can also prolong the survival time, especially with the steroid cyclosporine regimes.