|Raynauds Syndrome, Raynauds Disease
Raynauds 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. Raynauds disease resolves with warming.
Raynauds phenomenon may be idiopathic (Raynauds 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 Raynauds disease is most common in young women (60 to 90% of reported cases).
Raynauds 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 Raynauds. 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 Raynauds 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 Raynauds 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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