An Under-Recognized Manifestation of Systemic Sclerosis: Macrovascular Peripheral Arterial Disease

Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease characterized by skin and visceral fibrosis and by a well-known microangiopathy leading to Raynaud's phenomenon, digital ischemia, pulmonary arterial hypertension, and scleroderma renal crisis. By contrast, macrovascular involvement has been less extensively described and may be under-recognized. We report the case of a 66-year-old woman with a 16-year history of SSc. The diagnosis was based on cutaneous sclerosis, Raynaud's phenomenon complicated by digital necrosis, positive antinuclear antibodies, and interstitial lung disease with pulmonary fibrosis. She was admitted for intermittent claudication of the lower limbs. CT angiography of the lower extremities revealed diffusely small-caliber arteries with long, thread-like stenoses, consistent with macrovascular peripheral arterial involvement in the context of SSc. This case illustrates that SSc may be associated not only with microvascular but also with clinically significant macrovascular disease. Awareness of this complication and the use of vascular imaging in symptomatic patients are essential for early diagnosis and appropriate management.