Carotid thrombus formation and extension during anticoagulation: a case report of large vessel disease and hypercoagulable state in systemic sclerosis.

Systemic sclerosis (SSc) is an autoimmune multisystem disorder of connective tissue characterized by widespread vascular lesions and fibrosis. Limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dsSSC) are both subsets of SSc. The vascular component of SSc is an important part of the disease. Especially Raynaud's phenomenon and microcirculatory abnormalities are well recognized. Enhanced coagulation pathways, decreased fibrinolysis, and endothelial dysfunction probably contribute to vascular events in SSc. Macrovascular disease is not recognized as a major feature of SSc. However, several studies report an increase in large vessel disease and mortality rate attributable to cardiovascular causes. We present a patient with lcSSc with an acute embolic stroke due to a large carotid thrombus. A hypercoagulable state was suspected because of thrombus formation during oral anticoagulation and extension during intravenous heparin treatment. This is one of the few reports of large vessel disease in systemic sclerosis. The hypercoagulable state may be related to systemic sclerosis.