并发间质性肺炎的临床无肌病皮肌炎生存分析——13年数据回顾

Amyopathic dermatomyositis complicated by acute/subacute interstitial pneumonia: a 13-year retrospective analysis

目的:分析并发急性/亚急性间质性肺炎(acute/subacute interstitial pneumonia,A/SIP)的临床无肌病皮肌炎(clinically amyopathic dermatomyositis,CADM)患者的生存率变化及其影响因素。方法:回顾分析1998年1月至2010年12月,上海交通大学医学院附属仁济医院风湿免疫科收治的79例并发A/SIP的CADM患者,以其首次收治时间分为历史组和晚近组。采用Kaplan-Meier法进行生存分析,用COX回归分析预后的影响因素。结果:Kaplan-Meier生存分析显示,晚近组患者的半年生存率(63.8%)明显高于历史组(38.1%,P=0.023)。与历史组比较,晚近组生存状况的改善主要发生在SIP患者中(P=0.018)。单因素分析发现,晚近组中并发感染(P=0.019)和接受大剂量激素(P=0.018)治疗者较历史组少;而联合免疫抑制剂[免疫抑制剂包括环磷酰胺/霉酚酸酯/环孢素(P=0.0017)或单用环孢素(P=0.003)],先于低氧血症前使用者(P=0.006)的例数增加。COX回归分析发现,并发感染(RR=2.635,95%CI:1.091,6.364)、接受大剂量激素冲击(RR=3.109,95%CI:1.432,6.751)为预后不良因素,而免疫抑制剂先于低氧血症前使用[RR=0.435,95%CI:0.194,0.971)为预后有利因素。结论:本研究数据显示,早期及时联合中低剂量糖皮质激素和免疫抑制剂治疗有助于提高CADM-A/SIP患者的生存率,而合并感染是影响此类患者生存的危险因素。

Objective To analyze the survival change and prognostic factor in a cohort of patients with amyopathic dermatomyositis （CADM） complicated by acute/subacute interstitial pneumonia （MSIP）. Methods Seventy-nine CADM patients complicated by AIP/SIP during the past 13 years （Jan 1998 to Dee 2010）at Shanghai Renji Hospital were analyzed retrospectively. Patients were divided into two groups, the historical group and recent group with Jan 2007 as the cutoff time. Kaplan-Meier survival curve was performed and COX regression analysis was used to analyze the prognostic factors. Results Kaplan-Meier survival analysis showed that the half-year survival rate of patients in recent group （Jan 2007-Dec 2010） （63.8%） was significantly higher than that of patients in historical group （Jan 1998-Dec 2006） （38.1%, P=0.023）. The improvement of survival was mainly seen in CADM patients complicated by subacute interstitial pneumonia （SIP）（P=-0.018）. Univariate analysis indicated that the number of patients complicated by infection （P=0.019） or receiving large doses of corticosteroids （P=0.018） was significantly lower and the number of patients receiving immunosuppressants,CTX/MMF/CsA （P=0.0017） or CsA alone （P=0.003） and before the occurrence of hypoxemia （P=0.006） was significantly higher in recent group. COX regression analysis indicated that complicated by infection （RR = 2.635, 95% CI： 1.091, 6.364）,receiving high dose steroid pulse therapy （RR=3.109, 95% CI：1.432, 6.751） were independent risk factors for prognosis, while immunosuppressants taken before the occurrence of hypoxemia was a favorable prognostic factor （RR=0.435, 95% CI： 0.194, 0.971）. Conclusions The results in this study indicates that early use of low-medium dose corticosteroids combined with immunosuppressants could improve the survival of CADM patient complicated by A/SIP, and complicated by infection is a major risk factor for prognosis.