Department of Rheumatology and Immunology, University of Pécs, Medical School, Pécs, Hungary.
Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
Arthritis Res Ther. 2023 May 20;25(1):84. doi: 10.1186/s13075-023-03063-1.
Systemic sclerosis (SSc) is a multi-organ disease with impaired health-related quality of life (HRQoL). The EULAR SSc Impact of Disease (ScleroID) is a newly introduced SSc-specific patient-reported outcome to evaluate HRQoL in SSc.
To investigate the correlation between the ScleroID and the involvement of organ systems as well as disease activity/damage in a SSc cohort from a large tertiary care centre.
The ScleroID and clinical characteristics including internal organ involvement and hand function were investigated in 160 consecutive patients with SSc (median age 46 (43;56) years; diffuse cutaneous SSc 55%).
A strong correlation was found between the ScleroID and articular disease activity scores (DAS28-CRP, DAS28-ESR, CDAI, SDAI), a hand function performance test, the Hand Anatomy Index and muscle strength tests. Additionally, a strong significant correlation was discovered using instruments representing hand function and musculoskeletal disability including the Cochin Hand Function Scale, the Quick Questionnaire of the Disability of the Hands, Arms and the Shoulders and the Health Assessment Questionnaire Disability Index. A significant negative correlation was found between the ScleroID score and the 6-min walking test (6MWT) (rho - 0.444, p < 0.001). Clinically mild lung/heart disease did not show increased ScleroID values. The Mouth Handicap in the Scleroderma Scale and the University of California Los Angeles Scleroderma Clinical Trials Consortium gastrointestinal tract 2.0 also showed significant positive correlations to the ScleroID score (rho: 0.626, p < 0.001; rho: 0.646, p < 0.001, respectively). Patients experiencing oesophageal difficulties bore a significantly higher score compared to individuals with a normal functioning oesophagus (3.2/1.5;4.5/ vs. 2.2/1.0;3.2/, p = 0.011). Moreover, the ScleroID showed a significant positive correlation to the revised EUSTAR disease activity index and modified activity index.
In a large single-centre cohort, the previously described ScleroID-related findings were confirmed. Furthermore, several organ involvement-related functional and performance tests showed a good correlation to the ScleroID including the 6MWT and gastrointestinal-related complaints. Many aspects of musculoskeletal damage, overall disease activity, pain and fatigue were also well represented in the ScleroID, which efficiently reflects the impact of organ involvement, disease activity and functional damage.
系统性硬化症（SSc）是一种多器官疾病，会导致健康相关生活质量（HRQoL）受损。EULAR SSc 疾病影响（ScleroID）是一种新引入的 SSc 特异性患者报告结局，用于评估 SSc 中的 HRQoL。
在一家大型三级保健中心的 SSc 队列中，研究 ScleroID 与器官系统受累以及疾病活动/损伤之间的相关性。
对 160 例连续 SSc 患者（中位年龄 46（43；56）岁；弥漫性皮肤 SSc 55%）进行了 ScleroID 和临床特征调查，包括内脏器官受累和手部功能。
ScleroID 与关节疾病活动评分（DAS28-CRP、DAS28-ESR、CDAI、SDAI）、手部功能表现测试、手部解剖指数和肌肉力量测试之间存在很强的相关性。此外，使用代表手部功能和肌肉骨骼残疾的仪器，包括科钦手部功能量表、手部、手臂和肩部残疾快速问卷和健康评估问卷残疾指数，也发现了很强的显著相关性。ScleroID 评分与 6 分钟步行试验（6MWT）呈显著负相关（rho-0.444，p<0.001）。临床轻度肺部/心脏疾病并未显示出更高的 ScleroID 值。在硬皮病量表中的口腔障碍和加利福尼亚大学洛杉矶分校硬皮病临床试验联合会胃肠道 2.0 中，ScleroID 评分也显示出显著的正相关性（rho：0.626，p<0.001；rho：0.646，p<0.001）。与食管功能正常的个体相比，患有食管困难的患者评分显著更高（3.2/1.5；4.5/ vs. 2.2/1.0；3.2/，p=0.011）。此外，ScleroID 与修订后的 EUSTAR 疾病活动指数和改良活动指数呈显著正相关。
在一个大型单中心队列中，证实了先前描述的与 ScleroID 相关的发现。此外，几个与器官受累相关的功能和表现测试与 ScleroID 有良好的相关性，包括 6MWT 和胃肠道相关的抱怨。肌肉骨骼损伤的许多方面、整体疾病活动、疼痛和疲劳在 ScleroID 中也得到了很好的体现，它有效地反映了器官受累、疾病活动和功能损伤的影响。
