Abstract：
OBJECTIVE: The current knowledge about the role of comorbidities in systemic sclerosis (SSc) is limited. Therefore, the aim of this study was to evaluate the prevalence of comorbidities and their impact on disease activity and prognosis in the Systemic sclerosis PRogression INvestiGation (SPRING) registry.
METHODS: SSc patients from the SPRING registry, fulfilling the ACR/EULAR 2013 classification criteria, with complete data on baseline comorbidities were enrolled. The Charlson comorbidity index (CCI) was used to quantify the overall comorbidity burden. The disease activity was calculated using the revised EUSTAR activity index (AI). The impact of SSc features on CCI, the effect of CCI on SSc disease activity and mortality were tested with multivariable regression models.
RESULTS: Among 1910 SSc patients enrolled, 67.3 % had at least one comorbidity at baseline. The most frequent comorbidities were systemic arterial hypertension (23.7 %), osteoporosis (12.9 %) and dyslipidemia (11 %). The mean value of CCI score was 2.0 ± 1.8. When patients were grouped according to increasing levels of CCI, a clear separation in the distribution of SSc-related clinical features could be observed. Among over 900 patients with available follow-up, no association between baseline CCI and changes in disease activity was observed. Conversely, the risk of death over time was independently predicted by both CCI and AI.
CONCLUSIONS: Comorbidities and disease activity independently impact on the prognosis of SSc patients. This suggests that the management of comorbidities, together with the reduction of disease activity, is fundamental to improve patient survival.
METHODS: SSc patients from the SPRING registry, fulfilling the ACR/EULAR 2013 classification criteria, with complete data on baseline comorbidities were enrolled. The Charlson comorbidity index (CCI) was used to quantify the overall comorbidity burden. The disease activity was calculated using the revised EUSTAR activity index (AI). The impact of SSc features on CCI, the effect of CCI on SSc disease activity and mortality were tested with multivariable regression models.
RESULTS: Among 1910 SSc patients enrolled, 67.3 % had at least one comorbidity at baseline. The most frequent comorbidities were systemic arterial hypertension (23.7 %), osteoporosis (12.9 %) and dyslipidemia (11 %). The mean value of CCI score was 2.0 ± 1.8. When patients were grouped according to increasing levels of CCI, a clear separation in the distribution of SSc-related clinical features could be observed. Among over 900 patients with available follow-up, no association between baseline CCI and changes in disease activity was observed. Conversely, the risk of death over time was independently predicted by both CCI and AI.
CONCLUSIONS: Comorbidities and disease activity independently impact on the prognosis of SSc patients. This suggests that the management of comorbidities, together with the reduction of disease activity, is fundamental to improve patient survival.
摘要：
目的：目前关于系统性硬化症（SSc）中合并症的作用的知识有限。因此,本研究的目的是评估系统性硬化症早发性评估(SPRING)注册中合并症的患病率及其对疾病活动性和预后的影响.
方法：来自SPRING注册表的SSc患者，符合ACR/EULAR2013分类标准，纳入了基线合并症的完整数据.Charlson合并症指数（CCI）用于量化总体合并症负担。使用修订的EUSTAR活动指数（AI）计算疾病活动。用多元回归模型检验了SSc特征对CCI的影响，CCI对SSc疾病活动性和死亡率的影响。
结果：在1910名SSc患者中，67.3%的患者在基线时至少有一种合并症。最常见的合并症是全身性动脉高血压（23.7%），骨质疏松症（12.9%）和血脂异常（11%）。CCI评分平均值为2.0±1.8。当根据CCI水平的增加对患者进行分组时，可以观察到SSc相关临床特征的分布明显分离。在900多名有随访的患者中，未观察到基线CCI与疾病活动性变化之间的关联.相反，随时间推移的死亡风险由CCI和AI独立预测.
结论：合并症和疾病活动性独立影响SSc患者的预后。这表明合并症的管理，随着疾病活动的减少，是提高患者生存率的基础。
方法：来自SPRING注册表的SSc患者，符合ACR/EULAR2013分类标准，纳入了基线合并症的完整数据.Charlson合并症指数（CCI）用于量化总体合并症负担。使用修订的EUSTAR活动指数（AI）计算疾病活动。用多元回归模型检验了SSc特征对CCI的影响，CCI对SSc疾病活动性和死亡率的影响。
结果：在1910名SSc患者中，67.3%的患者在基线时至少有一种合并症。最常见的合并症是全身性动脉高血压（23.7%），骨质疏松症（12.9%）和血脂异常（11%）。CCI评分平均值为2.0±1.8。当根据CCI水平的增加对患者进行分组时，可以观察到SSc相关临床特征的分布明显分离。在900多名有随访的患者中，未观察到基线CCI与疾病活动性变化之间的关联.相反，随时间推移的死亡风险由CCI和AI独立预测.
结论：合并症和疾病活动性独立影响SSc患者的预后。这表明合并症的管理，随着疾病活动的减少，是提高患者生存率的基础。
