Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinérAIR-Sclérodermie study.
Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinérAIR-Sclérodermie study.
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DOI：
10.1093/rheumatology/ken488
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发表时间：
2009-03
期刊：
影响因子：
--
通讯作者：
ItinérAIR-Sclérodermie Study Investigators
中科院分区：
文献类型：
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作者：
Hachulla E;Carpentier P;Gressin V;Diot E;Allanore Y;Sibilia J;Launay D;Mouthon L;Jego P;Cabane J;de Groote P;Chabrol A;Lazareth I;Guillevin L;Clerson P;Humbert M;ItinérAIR-Sclérodermie Study Investigators
目标。这项纵向研究调查了多中心ItinéRair-Scrérodermie队列中无严重肺纤维化或严重左心疾病的SSc患者的存活率、风险因素和死亡原因。方法：研究方法。在3年的随访中，从调查人员或法国国家死亡记录中获得生命状态。死亡原因被归类为与SSC相关或其他。数据在37个月、死亡或失访时间进行审查，以较早者为准。生存估计采用Kaplan-Meier方法。采用COX模型进行多因素生存分析。结果。总共有546名患者被随访，平均持续时间为37个月，平均为1547人年。基线时，大多数患者为女性，患有LcSSc，平均年龄为54.9±13.0岁，平均持续时间为8.8±8.1年。共有47例死亡，3年生存率为91.1%，累计死亡率为3.04/100病人年；17例(32.2%)死于肺动脉高压，8例(17.1%)死于癌症。在基线的47例PAH患者中，20例在随访期间死亡，3年存活率为56.3%。在多变量分析中，PAH[危险比(HR)7.246]、首次症状年龄(HR 1.052)、SSC持续时间(HR 1.047/年)和Rodnan皮肤评分(HR 1.045)与死亡率增加相关。结论。这项为期3年的研究观察到的存活率和死亡率估计与以前的报告相当。PAH增加了SSc患者死亡率的HR，证明每年进行超声心动图筛查是合理的。
Objectives. This longitudinal study investigated survival, risk factors and causes of death in the multicentre ItinérAIR-Sclérodermie 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.
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影响因子：
1.6
作者：
Ferri, C;Valentini, G;Tirri, G
通讯作者：
Tirri, G
影响因子：
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de Groote, P.;Gressin, V.;Humbert, M.
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--
作者：
Hachulla, E;Gressin, V;Humbert, M
通讯作者：
Humbert, M