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系统性硬化症中多种疾病共存的预后及功能影响

The Prognostic and Functional Impact of Multimorbidity in Systemic Sclerosis.

作者信息

Fairley Jessica L, Hansen Dylan, Proudman Susanna, Sahhar Joanne, Ngian Gene-Siew, Apostolopoulos Diane, Walker Jennifer, Host Lauren V, Stevens Wendy, Nikpour Mandana, Ross Laura

机构信息

The University of Melbourne and St. Vincent's Hospital Melbourne, Melbourne, Australia.

St. Vincent's Hospital Melbourne, Melbourne, Australia.

出版信息

ACR Open Rheumatol. 2025 Apr;7(4):e70034. doi: 10.1002/acr2.70034.

DOI:10.1002/acr2.70034
PMID:40259666
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12012261/
Abstract

OBJECTIVE

Our objective was to define the frequency and impact of multimorbidity in systemic sclerosis (SSc).

METHOD

Australian Scleroderma Cohort Study participants meeting American College of Rheumatology/EULAR criteria were included. Charlson Comorbidity Index scores were calculated at each visit, with multimorbidity defined as scores ≥4. Generalized estimating equations were used to model longitudinal data in multivariable models including age, sex, subclass, interstitial lung disease, and pulmonary arterial hypertension status. Survival was analyzed using Cox hazard modeling.

RESULTS

Of 2,000 participants, 85% were female, 27% had diffuse SSc, and 20% had multimorbidity. Among those with multimorbidity, key comorbidities were hypertension (81%), dyslipidemia (67%), obstructive lung disease (50%), malignancy (49%), and ischemic heart disease (IHD) (40%). Multimorbidity was associated with worse survival (hazard ratio [HR] 1.57, 95% confidence interval [CI] 1.30-1.91, P < 0.01). Renal disease had the largest impact (HR 2.41, 95% CI 1.46-3.98, P < 0.01), followed by left ventricular dysfunction (HR 1.76, 95% CI 1.21-2.57, P < 0.01), anticoagulation (HR 1.64, 95% CI 1.28-2.08, P < 0.01), and IHD (HR 1.45, 95% CI 1.16-1.80, P < 0.01). In multivariable modeling, multimorbidity was associated with poorer physical function (regression coefficient [RC] +0.17 units, 95% CI 0.13-0.21, P < 0.01). Peripheral vascular disease had the largest impact on physical function (RC +0.26 units, 95% CI 0.18-0.34, P < 0.01), followed by left ventricular dysfunction (RC +0.23 units, 95% CI 0.14-0.33, P = 0.01), IHD (RC +0.22 units, 95% CI 0.17-0.28, P < 0.01), and obstructive lung disease (RC +0.19 units, 95% CI 0.14-0.24, P < 0.01).

CONCLUSION

Multimorbidity occurred in 20% of patients in a large SSc cohort and was an important determinant of both prognosis and physical function. Effective treatment of non-SSc morbidity may improve outcomes for patients with SSc. Graphical Abstract. *p-value <0.05. Multimorbidity defined as Charlson Comorbidity Index scores ≥4.

ABBREVIATIONS

CI (confidence interval), CKD (chronic kidney disease), COPD (chronic obstructive pulmonary disease), HAQ-DI (health assessment questionnaire disability index), HR (hazard ratio), LVEF (left ventricular ejection fraction), IHD (ischaemic heart disease), HR (hazard ratio), IQR (interquartile range), PVD (peripheral vascular disease), SSc (systemic sclerosis).

摘要

目的

我们的目的是确定系统性硬化症(SSc)中合并症的发生率及其影响。

方法

纳入符合美国风湿病学会/欧洲抗风湿病联盟(ACR/EULAR)标准的澳大利亚硬皮病队列研究参与者。每次就诊时计算查尔森合并症指数评分,合并症定义为评分≥4。使用广义估计方程对多变量模型中的纵向数据进行建模,这些模型包括年龄、性别、亚型、间质性肺疾病和肺动脉高压状态。使用Cox风险模型分析生存率。

结果

在2000名参与者中,85%为女性,27%患有弥漫性SSc,20%患有合并症。在合并症患者中,主要合并症为高血压(81%)、血脂异常(67%)、阻塞性肺疾病(50%)、恶性肿瘤(49%)和缺血性心脏病(IHD)(40%)。合并症与较差的生存率相关(风险比[HR]1.57,95%置信区间[CI]1.30 - 1.91,P < 0.01)。肾脏疾病的影响最大(HR 2.41,95% CI 1.46 - 3.98,P < 0.01),其次是左心室功能障碍(HR 1.76,95% CI 1.21 - 2.57,P < 0.01)、抗凝治疗(HR 1.64,95% CI 1.28 - 2.08,P < 0.01)和IHD(HR 1.45,95% CI 1.16 - 1.80,P < 0.01)。在多变量建模中,合并症与较差的身体功能相关(回归系数[RC]+0.17单位,95% CI 0.13 - 0.21,P < 0.01)。周围血管疾病对身体功能的影响最大(RC +0.26单位,95% CI 0.18 - 0.34,P < 0.01),其次是左心室功能障碍(RC +0.23单位,95% CI 0.14 - 0.33,P = 0.01),IHD(RC +0.22单位,95% CI 0.17 - 0.察尔森合并症指数评分≥4。

缩写

CI(置信区间)、CKD(慢性肾脏病)、COPD(慢性阻塞性肺疾病)、HAQ - DI(健康评估问卷残疾指数)、HR(风险比)、LVEF(左心室射血分数)、IHD(缺血性心脏病)、HR(风险比)IQR(四分位数间距)、PVD(周围血管疾病)、SSc(系统性硬化症)。 28,P < 0.01)和阻塞性肺疾病(RC +0.19单位,95% CI 0.14 - 0.24,P < 0.01)。

结论

在一个大型SSc队列中,20%的患者发生合并症,这是预后和身体功能的重要决定因素。有效治疗非SSc合并症可能改善SSc患者的预后。图摘要。*p值<0.05。合并症定义为查尔森合并症指数评分≥4。

缩写

CI(置信区间)、CKD(慢性肾脏病)、COPD(慢性阻塞性肺疾病)、HAQ - DI(健康评估问卷残疾指数)、HR(风险比)、LVEF(左心室射血分数)、IHD(缺血性心脏病)、HR(风险比)、IQR(四分位数间距)、PVD(周围血管疾病)、SSc(系统性硬化症) 。

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Towards a comprehensive approach to the management and prognosis of systemic sclerosis's patients: The role of comorbidities in the SPRING-SIR registry.迈向系统性硬化症患者管理与预后的综合方法:合并症在SPRING-SIR注册研究中的作用
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