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系统性硬化症相关间质性肺疾病:ScleroID研究的见解与局限

Systemic Sclerosis-Associated ILD: Insights and Limitations of ScleroID.

作者信息

Niță Cristina, Groșeanu Laura

机构信息

Faculty of Medicine, Carol Davila University of Medicine and Pharmacy, 050474 Bucharest, Romania.

Sfanta Maria Clinical Hospital, 011172 Bucharest, Romania.

出版信息

Diagnostics (Basel). 2026 Jan 4;16(1):158. doi: 10.3390/diagnostics16010158.

Abstract

Pulmonary involvement in systemic sclerosis (SSc) is typically assessed using pulmonary function tests (PFTs), high-resolution CT (HRCT), and composite indices. Patient-reported outcomes (PRO), including ScleroID, provide insight into quality of life, but their relationship with clinical measures and role in overall disease assessment remain unclear. To assess the correlation between ScleroID scores and both lung involvement and disease activity/damage in a cohort of SSc-ILD patients from a large tertiary care center. : Disease activity [European Scleroderma Study Group Activity Index (EScSG-AI), Scleroderma Clinical Trials Consortium Activity Index (SCTC-AI)], disease severity [Medsger severity scale (MSS)], and PRO measure ScleroID were assessed for associations with the extent and severity of SSc-ILD. In 82 patients with SSc-ILD (mean age 56.0 ± 10.8 years; median disease duration 4.2 ± 4.7 years), higher fibrosis extent (>20%) was associated with worse lung function, greater exercise limitation, and higher ScleroID scores, particularly in fatigue, social life, and body mobility domains (all ≤ 0.03). Patients with >20% fibrosis also had worse NYHA class and Borg scores during 6-MWD ( < 0.001). Cross-sectional correlations showed that ScleroID total and individual domains were negatively associated with FVC% and 6-MWD, and positively with ILD extent on HRCT. Fatigue, social impact, and mobility domains correlated most strongly with disease activity and severity scores, especially in patients with > 20% fibrosis (r = 0.384-0.635, all ≤ 0.016), whereas breathlessness showed minimal associations (r < 0.2). In SSc-ILD, greater lung fibrosis and functional impairment are associated with worse patient-reported quality of life, particularly in fatigue, mobility, and social domains. ScleroID scores reflect both physiological severity and disease burden highlighting its value as a multidimensional outcome measure in patients with more advanced disease.

摘要

系统性硬化症(SSc)的肺部受累情况通常通过肺功能测试(PFTs)、高分辨率CT(HRCT)和综合指数进行评估。患者报告的结局(PRO),包括ScleroID,能提供有关生活质量的见解,但其与临床指标的关系以及在整体疾病评估中的作用仍不明确。为了评估来自一个大型三级护理中心的一组系统性硬化症相关间质性肺病(SSc-ILD)患者中ScleroID评分与肺部受累及疾病活动/损伤之间的相关性。:评估疾病活动度[欧洲硬皮病研究组活动指数(EScSG-AI)、硬皮病临床试验联盟活动指数(SCTC-AI)]、疾病严重程度[梅兹格严重程度量表(MSS)]以及PRO指标ScleroID与SSc-ILD的范围和严重程度之间的关联。在82例SSc-ILD患者中(平均年龄56.0±10.8岁;疾病中位病程4.2±4.7年),更高的纤维化程度(>20%)与更差的肺功能、更大的运动受限以及更高的ScleroID评分相关,尤其是在疲劳、社交生活和身体活动领域(均≤0.03)。纤维化程度>20%的患者在6分钟步行试验(6-MWD)期间的纽约心脏协会(NYHA)分级和博格评分也更差(<0.001)。横断面相关性分析显示,ScleroID总分及各个领域与用力肺活量百分比(FVC%)和6-MWD呈负相关,与HRCT上的间质性肺病范围呈正相关。疲劳、社会影响和活动领域与疾病活动度和严重程度评分的相关性最强,尤其是在纤维化程度>20%的患者中(r = 0.384 - 0.635,均≤0.016),而呼吸困难的相关性最小(r < 0.2)。在SSc-ILD中,更严重的肺纤维化和功能损害与更差的患者报告生活质量相关,尤其是在疲劳、活动和社交领域。ScleroID评分既反映了生理严重程度又反映了疾病负担,凸显了其作为更晚期疾病患者多维结局指标的价值。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e4a7/12785287/89baa673fdbc/diagnostics-16-00158-g001a.jpg

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