Introduction Central nervous system involvement in scleroderma has traditionally been considered uncommon. Recent studies suggest that scleroderma might be associated with an increased risk of cerebrovascular disease (CBVD), independent of conventional cardiovascular risk factors. We present a case series and a systematic review to capture the spectrum of CBVD in scleroderma, through a detailed description of clinical, demographic, laboratory, and radiographical findings. Methods In our case series, we included consecutive patients with scleroderma and CBVD seen over 35 years by our group in different hospitals in the United States. We also performed a systematic review from inception to July 2022. MEDLINE/EMBASE/WoS were searched for "scleroderma", "systemic scleroderma", "systemic sclerosis", "cerebrovascular", "stroke", "cerebrovascular disorders", "cerebrovascular disease". Results Fourteen patients with scleroderma and CBVD were included in our case series (mean age 48-year, 85% female). CBVDs were ischemic stroke (64%), hemorrhagic stroke (7%), venous thrombosis (7%), ischemic optic neuropathy (7%), probable ischemic stroke (14%). Of the 110 studies identified in our systematic review (45,484 patients), 82 reports with patient level data were included for quantitative analysis (93 patients, mean age 48-year, 79% female). Despite 16 different CBVD types identified, ischemic stroke was the most common CBVD (29%), followed by vasculopathy (20%), hemorrhage (12%), vasculitis (11%), and intracranial aneurysm (11%). Discussion/Conclusion Our relatively large case series combined with a systematic review of CBVD in SCL patients shows a heterogeneous spectrum of CBVD etiology, with acute ischemic stroke being the most common in our cases and in our literature review. A complex interaction between chronic inflammation, autoimmune mechanisms, and endothelial dysfunction seems to underlie the CBVD heterogeneity in scleroderma patients. This review informs clinicians about the spectrum of CBVD related to scleroderma and raise awareness about scleroderma being a possible risk factor for early onset CBVD.

中文翻译：

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硬皮病脑血管疾病谱：病例系列和系统评价。

简介 传统上认为硬皮病累及中枢神经系统的情况并不常见。最近的研究表明，硬皮病可能与脑血管疾病（CBVD）风险增加有关，与传统的心血管危险因素无关。我们通过对临床、人口统计学、实验室和放射学检查结果的详细描述，提出了一个病例系列和系统评价，以捕获硬皮病中的 CBVD 谱。方法 在我们的病例系列中，我们纳入了我们小组在美国不同医院就诊超过 35 年的连续患有硬皮病和 CBVD 的患者。我们还进行了从开始到2022年7月的系统评价。MEDLINE/EMBASE/WoS搜索了“硬皮病”、“系统性硬皮病”、“系统性硬化症”、“脑血管”、“中风”、“脑血管疾病”、“脑血管疾病” ”。结果 我们的病例系列中纳入了 14 名患有硬皮病和 CBVD 的患者（平均年龄 48 岁，85% 为女性）。CBVD 包括缺血性中风 (64%)、出血性中风 (7%)、静脉血栓形成 (7%)、缺血性视神经病变 (7%)、疑似缺血性中风 (14%)。在我们的系统评价中确定的 110 项研究（45,484 名患者）中，82 份包含患者水平数据的报告被纳入定量分析（93 名患者，平均年龄 48 岁，79% 为女性）。尽管已确定 16 种不同的 CBVD 类型，但缺血性中风是最常见的 CBVD (29%)，其次是血管病变 (20%)、出血 (12%)、血管炎 (11%) 和颅内动脉瘤 (11%)。讨论/结论 我们相对较大的病例系列结合对 SCL 患者 CBVD 的系统评价显示 CBVD 病因的异质性，其中急性缺血性卒中是我们的病例和文献综述中最常见的。慢性炎症、自身免疫机制和内皮功能障碍之间复杂的相互作用似乎是硬皮病患者 CBVD 异质性的基础。本综述向临床医生介绍了与硬皮病相关的 CBVD 范围，并提高了人们对硬皮病作为早发 CBVD 可能危险因素的认识。