Abstract
RATIONALE: 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. PATIENT CONCERNS: 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. DIAGNOSES: 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. INTERVENTIONS AND OUTCOMES: 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. LESSONS: 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.