Berro Mohammad, Tawashi Kenana, Sharaf Mohammad, Ibrahim Lin, Abdulaziz Osama
Faculty of Medicine, Damascus University, Damascus, Syria.
Al Mouwasat University Hospital, Damascus University, Damascus, Syria.
Medicine (Baltimore). 2025 Sep 5;104(36):e44331. doi: 10.1097/MD.0000000000044331.
Systemic sclerosis (SS) is an immune-mediated connective disease characterized by skin fibrosis, microvascular damage, and multisystem manifestations. One of the most important processes in connective tissue disorders is vasculitis. The clinical findings can differ when the disease is presented with an antineutrophil cytoplasmic antibody. Macrovascular disease in SS patients is rare, while the heart is the most affected organ in this condition; cerebrovascular involvement is sporadic.
A 46-year-old male presented with left-sided hemiparesis, accompanied by numbness, tingling, and blurred vision in the right eye. These symptoms lasted several minutes before resolving and had occurred more than 10 times in the last month. The patient was diagnosed with SS, Sjögren's disease, and hypertension 3 months ago after 15 years of symptoms accompanied by SS. The clinical examination revealed stiffened skin, predominantly on the face and hands, as well as generalized dullness of respiratory sounds. The examination of the nervous system was normal.
A color Doppler ultrasound of the carotid arteries indicated 90% stenosis of the right internal carotid artery. A multisegment computed tomography scan of the aortic arch, branches, and carotid arteries showed many interruptions in several arteries. Magnetic resonance imaging of the brain showed multiple high-signal foci and plaques, which may indicate the presence of vascular lesions. Therefore, the patient was diagnosed with an ischemic cerebrovascular accident secondary to SS.
Treatment included aspirin, clopidogrel, atorvastatin calcium, azathioprine, prednisone, and hypertension medications. The patient was referred to the Department of Vascular Surgery for stent placement or bypass surgery. During follow-up, the patient developed 4 additional ischemic strokes similar to the previous episodes.
This case highlights a patient with SS who presented with macrovascular stenosis. This unusual presentation underscores the importance of vigilance for macrovascular involvement in SS patients presenting with neurological symptoms.
系统性硬化症（SS）是一种免疫介导的结缔组织疾病，其特征为皮肤纤维化、微血管损伤和多系统表现。结缔组织疾病中最重要的过程之一是血管炎。当疾病伴有抗中性粒细胞胞浆抗体时，临床表现可能会有所不同。SS患者的大血管疾病很少见，而心脏是这种情况下受影响最严重的器官；脑血管受累较为散在。
一名46岁男性出现左侧偏瘫，伴有右眼麻木、刺痛和视力模糊。这些症状持续数分钟后缓解，且在过去一个月内发生了10多次。该患者在出现SS症状15年后，于3个月前被诊断为SS、干燥综合征和高血压。临床检查发现皮肤变硬，主要集中在面部和手部，同时呼吸音普遍减弱。神经系统检查正常。
颈动脉彩色多普勒超声显示右侧颈内动脉狭窄90%。主动脉弓、分支和颈动脉的多段计算机断层扫描显示多条动脉有多处中断。脑部磁共振成像显示多个高信号灶和斑块，这可能表明存在血管病变。因此，该患者被诊断为继发于SS的缺血性脑血管意外。
治疗包括阿司匹林、氯吡格雷、阿托伐他汀钙、硫唑嘌呤、泼尼松和高血压药物。患者被转诊至血管外科进行支架置入或搭桥手术。在随访期间，患者又发生了4次与之前发作类似的缺血性中风。
本病例突出了一名患有大血管狭窄的SS患者。这种不寻常的表现强调了对出现神经症状的SS患者警惕大血管受累的重要性。
