RaynaudÕs disease is a bilateral vasospastic disorder manifested by intermittent attacks of extreme pallor, then cyanosis of the fingers (rarely, of the toes) brought on by cold exposure. It may accompany emotional upset. With warming, vasodilatation and intense redness develops, followed by swelling, throbbing, paresthesias. RaynaudÕs disease resolves with warming.
RaynaudÕs phenomenon may be idiopathic (RaynaudÕs disease) or secondary to other conditions, including: connective tissue disorders (e.g., scleroderma, RA, SLE), obstructive arterial diseases (arteriosclerosis obliterans, thromboangiitis obliterans, thoracic outlet syndrome), neurogenic lesions, drug intoxications (ergot and methysergide), dysproteinemias, myxedema, primary pulmonary hypertension, and trauma. Idiopathic RaynaudÕs disease is most common in young women (60 to 90% of reported cases).
RaynaudÕs syndrome may involve increased sensitivity of alpha-2-adrenergic receptors in digital vessels in the primary type. Serotonin receptors (5-HT2 type) may be involved in secondary RaynaudÕs. Platelet and blood viscosity abnormalities are also implicated. Current research focuses on prostaglandin metabolism, microcirculation, and the role of the endothelial cells. The clinical association between RaynaudÕs phenomenon and migraine headaches, variant angina, and pulmonary hypertension suggests that there is a common mechanism for vasospasm. The threshold for the vasospastic response in RaynaudÕs disease is lowered by anything that activates sympathetic outflow or releases catecholamines (e.g., emotion) in addition to local cold.
Risk factors include smoking and an existing autoimmune or connective tissue disorder.
Lab tests are used to screen for underlying secondary causes (CBC, ESR, RA, ANA, immunoelectrophoresis, esophageal motility studies). A cold challenge test will elicit characteristic color changes in hands. Nailfold capillaroscopy is used to detect enlarged, irregular capillary loops of other connective tissue diseases (primary Raynaud's should show normal vasculature).
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