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首页> 外文期刊>Rheumatology >Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinerAIR-Sclerodermie study.
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Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinerAIR-Sclerodermie study.

机译：系统性硬化症患者死亡和3年生存的危险因素：法国ItinerAIR-Sclerodermie研究。

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OBJECTIVES: This longitudinal study investigated survival, risk factors and causes of death in the multicentre ItinerAIR-Sclerodermie cohort of patients with SSc without severe pulmonary fibrosis or severe left heart disease at baseline. METHODS: At 3-year follow-up, vital status was obtained from investigators or French national death records. Causes of death were classified as SSc-related or otherwise. Data were censored at 37 months, time of death or loss to follow-up, whichever was earlier. Survival was estimated using the Kaplan-Meier method. Multivariate survival analyses were conducted using the Cox model. RESULTS: In total, 546 patients were followed for a median duration of 37 months, representing 1547 patient-years. At baseline, the majority of patients were female, with lcSSc, mean age 54.9 +/- 13.0 years and mean duration of SSc of 8.8 +/- 8.1 years. In total, 47 patients died, giving a 3-year survival of 91.1% and cumulative mortality of 3.04 deaths per 100 patient-years; 17 deaths (32.2%)resulted from pulmonary arterial hypertension (PAH) and eight (17.1%) from cancer. Of the 47 patients with PAH at baseline, 20 died during follow-up, giving a 3-year survival of 56.3%. In a multivariate analysis, PAH [hazard ratio (HR) 7.246], age at first symptom (HR 1.052), duration of SSc (HR 1.047 per year) and Rodnan skin score (per one point) (HR 1.045) were associated with increased mortality. CONCLUSION: This 3-year study observed survival and mortality estimates that were comparable with previous reports. PAH increased the HR for mortality in patients with SSc, justifying yearly echocardiographic screening.
机译：目的：这项纵向研究调查了基线时无严重肺纤维化或严重左心病的SSc多中心ItinerAIR-Sclerodermie队列的生存率，危险因素和死亡原因。方法：在3年的随访中，从研究者或法国国民死亡记录中获得了生命状态。死亡原因归类为与SSc相关或其他原因。数据在37个月（死亡或失访时间）进行检查，以较早者为准。使用Kaplan-Meier方法估算生存率。使用Cox模型进行多变量生存分析。结果：总共546例患者被随访，平均持续时间为37个月，代表1547病人年。在基线时，大多数患者为女性，患有lcSSc，平均年龄为54.9 +/- 13.0岁，平均SSc病程为8.8 +/- 8.1年。总共有47例患者死亡，3年生存率为91.1％，每100个患者-年的累积死亡率为3.04例死亡。肺动脉高压（PAH）导致17例死亡（32.2％），癌症致死8例（17.1％）。在基线时的47位PAH患者中，有20位在随访期间死亡，3年生存率为56.3％。在多变量分析中，PAH [危险比（HR）7.246]，首次出现症状的年龄（HR 1.052），SSc持续时间（每年HR 1.047）和Rodnan皮肤评分（每1分）（HR 1.045）与增加死亡。结论：这项为期3年的研究观察到的生存和死亡率估计与以前的报告相当。 PAH可提高SSc患者死亡率的HR，因此有必要每年进行超声心动图筛查。

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