Systemic Vasospasm

Raynaud phenomenon (RP) is thought to be an exaggerated vasoactive response to environmental temperatures and emotional stress. Abnormal vascular reactivity is also the presumed mechanism underlying several common and uncommon disorders such as migraine headaches, preeclampsia, and vasospastic angina. While epidemiologic studies (case reports, case series, and some controlled studies) have linked RP and these conditions, there is yet to be any clearly defined systemic vasoreactivity syndrome. Raynaud phenomenon is also a hallmark feature of systemic sclerosis, a disease with components of both vasospasm and structural small vessel disease that may lead to tissue ischemia of the digits. Whether other organ systems in patients with scleroderma also have a component of vasospasm as either an inciting event or contributing factor to damage is yet to be clearly defined. This chapter first examines the possible role of vasospasm in scleroderma outside of the peripheral circulation and RP in playing a role in the pathogenesis of scleroderma renal crisis, pulmonary hypertension, and cardiac disease. This chapter then examines the evidence that may exist linking RP with systemic vascular disorders.