Systemic sclerosis associated with moyamoya syndrome: A case report and literature review

Chen, Haifeng; Jiang, Xinyu; Shi, Yueqin; Yuan, Fenghong; Hu, Zhigang

doi:10.1016/j.imbio.2019.11.017

Cited by 5 publications

(2 citation statements)

References 12 publications

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“…With our systematic review, we identified 4 patients with SSc and angiographically proven Moya-Moya syndrome. The term "MoSys" syndrome has been suggested to describe the phenomenon of Moya-Moya syndrome with underlying SCL [30]. Several mechanisms have been hypothesized, including vasculitis and endothelial hyperplasia.…”

Section: Vasculitis and Vasculopathymentioning

confidence: 99%

“…Several mechanisms have been hypothesized, including vasculitis and endothelial hyperplasia. It is thought that a diagnosis of peripheral vascular disease in patients with SCL may be a harbinger of future Moya-Moya syndrome, and early MR angiography screening may be beneficial in identifying and addressing progressive vaso-occlusive disease using optimal management [30]. No designated protocols for prevention or treatment are available given the rarity of this association.…”

Section: Vasculitis and Vasculopathymentioning

confidence: 99%

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Spectrum of Cerebrovascular Disease in Scleroderma: A Case Series and Systematic Review

Rosso,

Ramaswamy,

Aharonoff

et al. 2023

Cerebrovasc Dis

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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 USA. 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 years, 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 years, 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%). 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.

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Section: Vasculitis and Vasculopathymentioning

confidence: 99%

Section: Vasculitis and Vasculopathymentioning

confidence: 99%