Carotid Artery Stenting for Symptomatic Severe Carotid Artery Stenosis in a Patient with Systemic Sclerosis (Scleroderma)

Systemic sclerosis (SSc) is a systemic autoimmune disease of unknown etiology characterized by three hallmarks: vasculopathy with microvascular involvement, fibrosis of the skin and visceral organs, and systemic inflammation characterized by the presence of circulating autoantibodies and proinflammatory cytokines. A growing body of evidence suggests that patients with scleroderma have significantly elevated risk of ischemic stroke, and accelerated carotid atherosclerosis appears to be strongly associated with excessive cerebrovascular complications. A 71-year-old previously independent woman with a history of scleroderma and arterial hypertension under medical control was brought to the emergency room because of a left-hemispheric transient ischemic attack of 10-min duration, which manifested with speech disturbance and numbness in the right hand. The patient had experienced three similar episodes during the week before admission. Her National Institutes of Health Stroke Scale (NIHSS) score upon admission was 0 points. Her admission noncontrast cranial CT was unremarkable. CT angiography showed a long, heavily calcified, irregular atherosclerotic lesion at the left common carotid artery bifurcation, causing its severe stenosis. The patient received a loading dose of aspirin and clopidogrel, and the VerifyNow system was used for platelet reactivity testing. She then underwent an uneventful stent-assisted left internal carotid artery angioplasty and stenting procedure. Her clinical course was unremarkable, and she was discharged within 3 days after the intervention with an NIHSS score of 0 and a modified Rankin Score (mRS) of 0 points. This chapter describes ischemic stroke caused by large artery atherosclerosis in patients with scleroderma, with a focus on pathophysiological mechanisms and therapeutic options.