Orlandi Martina, Bellando-Randone Silvia, De Angelis Rossella, Ferri Clodoveo, Giuggioli Dilia, Cacciapaglia Fabio, Magnani Luca, Cuomo Giovanna, Gigante Antonietta, Codullo Veronica, Campochiaro Corrado, Ariani Alarico, Foti Rosario, Guiducci Serena, Matucci-Cerinic Marco, Bruni Cosimo
Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence & Division of Rheumatology AOUC, University of Florence, Florence, Italy; University Hospital of Modena and Reggio Emilia School of Medicine, Department of Medical and Surgical Sciences for Children and Adults, Modena, Italy.
Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence & Division of Rheumatology AOUC, University of Florence, Florence, Italy.
Eur J Intern Med. 2024 Dec;130:130-136. doi: 10.1016/j.ejim.2024.07.040. Epub 2024 Aug 14.
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.
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.
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.
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.
目前关于合并症在系统性硬化症（SSc）中作用的知识有限。因此，本研究的目的是在系统性硬化症进展调查（SPRING）登记处评估合并症的患病率及其对疾病活动和预后的影响。
纳入SPRING登记处符合2013年美国风湿病学会/欧洲抗风湿病联盟（ACR/EULAR）分类标准且有完整基线合并症数据的SSc患者。使用Charlson合并症指数（CCI）量化总体合并症负担。使用修订后的欧洲抗硬皮病研究组活动指数（AI）计算疾病活动度。通过多变量回归模型测试SSc特征对CCI的影响、CCI对SSc疾病活动和死亡率的影响。
在纳入的1910例SSc患者中，67.3%在基线时有至少一种合并症。最常见的合并症是系统性动脉高血压（23.7%）、骨质疏松症（12.9%）和血脂异常（11%）。CCI评分的平均值为2.0±1.8。当根据CCI水平升高对患者进行分组时，可以观察到SSc相关临床特征分布的明显差异。在900多名有随访数据的患者中，未观察到基线CCI与疾病活动度变化之间的关联。相反，CCI和AI均可独立预测随时间推移的死亡风险。
合并症和疾病活动度独立影响SSc患者的预后。这表明合并症的管理以及疾病活动度的降低对于提高患者生存率至关重要。
