Cutolo Maurizio, Vojinovic Tamara, Paolino Sabrina, Campitiello Rosanna, Smith Vanessa
Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Genoa, Italy.
IRCCS Ospedale Policlinico San Martino, Genoa, Italy.
ACR Open Rheumatol. 2025 Apr;7(4):e70032. doi: 10.1002/acr2.70032.
Systemic sclerosis (SSc) is a chronic autoimmune rheumatic disease characterized by vascular damage, immune system dysregulation and fibrosis. The hallmark features include microvascular alterations and progressive tissue fibrosis, affecting skin, internal organs as well central and peripheral nervous system, adding to the disease's complexity and influencing overall outcomes. Of note, SSc has also been linked to macrovascular and cardiovascular involvement, including cerebrovascular damage as observed in stroke. Indeed, advanced neuroimaging is highly recommended for assessing cerebrovascular status in overt SSc to evaluate the complex interactions between cerebrovascular dysfunction and brain tissue damage and/or inflammation. Cerebral vasospasm detected by angiography, as well as an increase in subclinical cerebrovascular atherosclerosis observed by ultrasonography (carotid intimal medial thickness), are predictive for elevated stroke risk. Furthermore, a significant brain hypoperfusion detected by magnetic resonance imaging, along with white matter focal and/or diffuse signal abnormalities in SSc, have been found associated with concomitant peripheral microvascular damage detectable by "Active" and "Late" nail fold video capillaroscopy scleroderma patterns. Finally, the presence of calcifications in small arteries and arterioles found postmortem in the brain of SSc patients reinforces the hypothesis that SSc is associated with brain vascular remodeling. Furthermore, the current state of art shows an increased risk of cerebrovascular events in the SSc, confirmed by neuroimaging. Given the lack of updated comprehensive reviews on cerebrovascular involvement in SSc, we gathered the most relevant evidence on central nervous system damage, highlighting the underlying mechanisms, clinical implications, and potential advantages that neuroimaging may provide for its early detection.
系统性硬化症（SSc）是一种慢性自身免疫性风湿性疾病，其特征为血管损伤、免疫系统失调和纤维化。标志性特征包括微血管改变和进行性组织纤维化，可累及皮肤、内脏器官以及中枢和外周神经系统，增加了疾病的复杂性并影响总体预后。值得注意的是，SSc还与大血管和心血管受累有关，包括中风时观察到的脑血管损伤。事实上，强烈建议对明显的SSc患者进行高级神经影像学检查，以评估脑血管状况，从而评估脑血管功能障碍与脑组织损伤和/或炎症之间的复杂相互作用。血管造影检测到的脑血管痉挛，以及超声检查（颈动脉内膜中层厚度）观察到的亚临床脑血管动脉粥样硬化增加，可预测中风风险升高。此外，磁共振成像检测到的明显脑灌注不足，以及SSc患者白质局灶性和/或弥漫性信号异常，已被发现与通过“活动期”和“晚期”甲襞视频毛细血管镜检查硬皮病模式可检测到的外周微血管损伤相关。最后，在SSc患者大脑尸检中发现小动脉和小动脉中有钙化，这进一步支持了SSc与脑血管重塑相关的假说。此外，目前的技术水平表明，经神经影像学证实，SSc患者发生脑血管事件的风险增加。鉴于缺乏关于SSc患者脑血管受累的最新综合综述，我们收集了关于中枢神经系统损伤的最相关证据，强调了潜在机制、临床意义以及神经影像学在早期检测方面可能提供的潜在优势。
