Department of Diagnostic Imaging, Area of Cardiovascular and Interventional Imaging, Abruzzo Health Unit 1, Via Saragat -località Campo di Pile, 67100, L'Aquila, Italy.
SIRM Foundation, Italian Society of Medical and Interventional Radiology (SIRM), 20122, Milan, Italy.
Sci Rep. 2022 Mar 24;12(1):5125. doi: 10.1038/s41598-022-09064-5.
Systemic sclerosis (SSc) is an uncommon autoimmune disease. Aim of the study was to detect the occult cardiac involvement in asymptomatic SSc patients of recent onset (indicative of a more aggressive disease) with unenhanced Cardiac Magnetic Resonance (CMR). Our historical prospective study included naïve SSc patients of recent onset. Modified Rodnan Skin Score (mRSS) and Scleroderma Clinical Trial Consortium Damage Index (SCTC-DI) were calculated. Cardiac volumes and global myocardial strain were assessed and also compared with healthy group values. Pericardial involvement was further recorded. Thirty-one patients met inclusion criteria (54 ± 12 years; 1 M). Mean duration of disease was 6.8 years. All patients showed preserved systolic function. Higher incidence of pericardial involvement was founded in patients with disease accrual damage (OR: 9.6, p-value 0.01). Radial and longitudinal strain values resulted significantly different between healthy and SSc patients. GRS and GLS showed an independent predictive validity on damage accrual (HR: 1.22 and 1.47, respectively). Best C-index for disease progression was reached when strain values and pericardial evaluation were added to conventional risk factors (0.97, p-value: 0.0001). Strain analysis by CMR-TT may show a high capability both in identifying early cardiac involvement and stratifying its clinical aggressiveness, regardless of the standard damage indices and CMR contrast-dependent biomarker.
系统性硬化症(SSc)是一种罕见的自身免疫性疾病。本研究旨在检测近期发病(提示疾病更具侵袭性)的无症状 SSc 患者隐匿性心脏受累,这些患者未经增强型心脏磁共振(CMR)检查。我们的历史前瞻性研究纳入了近期发病的未经治疗的 SSc 患者。计算改良 Rodnan 皮肤评分(mRSS)和硬皮病临床试验联盟损害指数(SCTC-DI)。评估心脏容积和整体心肌应变,并与健康组进行比较。进一步记录心包受累情况。31 名患者符合纳入标准(54±12 岁;1 名男性)。疾病的平均病程为 6.8 年。所有患者均表现出保留的收缩功能。在疾病进展导致损害的患者中,心包受累的发生率更高(OR:9.6,p 值为 0.01)。健康患者和 SSc 患者之间的径向和纵向应变值存在显著差异。GRS 和 GLS 对损害进展具有独立的预测价值(HR:分别为 1.22 和 1.47)。当将应变值和心包评估添加到常规危险因素中时,疾病进展的最佳 C 指数最高(0.97,p 值:0.0001)。CMR-TT 的应变分析可能具有很高的能力,既能识别早期心脏受累,又能分层其临床侵袭性,而与标准损害指数和 CMR 对比依赖生物标志物无关。