Raynaud's syndrome is vasospasm of parts of the hand in response to cold or emotional stress, causing reversible discomfort and color changes (pallor, cyanosis, erythema, or a combination) in one or more digits. Occasionally, other acral parts (eg, nose, tongue) are affected. The disorder may be primary or secondary. Diagnosis is clinical; testing focuses on distinguishing primary from secondary disease. Treatment of uncomplicated cases includes avoidance of cold, biofeedback, smoking cessation, and, as needed, vasodilating Ca channel blockers (eg, nifedipine) or prazosin.

Overall prevalence is about 3 to 5%; women are affected more than men, and younger people are affected more than older people. Raynaud's syndrome is probably due to an exaggerated α₂-adrenergic response that triggers vasospasm; the mechanism is not defined.

Primary Raynaud's syndrome is much more common (> 80% of cases) than secondary; it occurs without symptoms or signs of other disorders. In the remaining 20% of patients with Raynaud's symptoms, a causative underlying disease (eg, systemic sclerosis) will be evident at initial presentation or diagnosed subsequently.

Secondary Raynaud's syndrome accompanies various disorders and conditions, mostly connective tissue disorders (see Table 1: Peripheral Arterial Disorders: Causes of Secondary Raynaud's Syndrome).

Table 1
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Causes of Secondary Raynaud's Syndrome Cause Examples Connective tissue disorders Mixed or undifferentiated connective tissue disease Polymyositis/dermatomyositis RA Sjögren's syndrome SLE Systemic sclerosis Endocrine disorders Hypothyroidism Hematologic disorders Cold agglutinin disease Polycythemia vera Neoplastic disorders Carcinoid Paraneoplastic syndrome Neurologic disorders Carpal tunnel syndrome Trauma Frost bite Vibration Vascular disorders Thoracic outlet syndrome Drugs β-Blockers Cocaine Ergot preparations Nicotine Sympathomimetic drugs

Nicotine commonly contributes to it but is often overlooked. Raynaud's syndrome may accompany migraine headaches, variant angina, and pulmonary hypertension, suggesting that these disorders share a common vasospastic mechanism.

Symptoms and Signs

Sensations of coldness, burning pain, paresthesias, or intermittent color changes of one or more digits are precipitated by exposure to cold, emotional stress, or vibration. All can be reversed by removing the stimulus. Rewarming the hands accelerates restoration of normal color and sensation.

Color changes are clearly demarcated across the digit. They may be triphasic (pallor, followed by cyanosis and after warming by erythema due to reactive hyperemia), biphasic (cyanosis, erythema), or uniphasic (pallor or cyanosis only). Changes are often symmetric. Raynaud's syndrome does not occur proximal to the metacarpophalangeal joints; it most commonly affects the middle 3 fingers and rarely affects the thumb. Vasospasm may last minutes to hours but is rarely severe enough to cause tissue loss.

Raynaud's syndrome secondary to a connective tissue disorder may progress to painful digital gangrene; Raynaud's syndrome secondary to systemic sclerosis tends to cause extremely painful, infected ulcers on the fingertips.

Diagnosis

• Clinical criteria
• Examination and testing for underlying disorder

Raynaud's syndrome itself is diagnosed clinically. Acrocyanosis (see Peripheral Arterial Disorders: Acrocyanosis) also causes color change of the digits in response to cold but differs from Raynaud's in that it is persistent, not easily reversed, and does not cause trophic changes, ulcers, or pain.

Primary and secondary forms are distinguished clinically, supported by vascular laboratory studies and blood testing. Vascular laboratory testing includes digital pulse wave forms and pressures. The primary blood testing is the panel for collagen vascular diseases.

Clinical findings

A thorough history and physical examination directed at identifying a causative disorder are helpful but rarely diagnostic.

Findings suggesting primary Raynaud's syndrome are the following:

• Age at onset < 40 (in 2/3 of cases)
• Mild symmetric attacks affecting both hands
• No tissue necrosis or gangrene
• No history or physical findings suggesting another cause

Findings suggesting secondary Raynaud's syndrome are the following:

• Age at onset > 30
• Severe painful attacks that may be asymmetric and unilateral
• Ischemic lesions
• History and findings suggesting an accompanying disorder

Laboratory testing

Blood tests (eg, measurement of ESR, antinuclear antibodies, rheumatoid factor, anticentromere antibody, anti-SCL-70 antibody) are done to detect accompanying disorders.

Treatment

• Trigger avoidance
• Smoking cessation
• Ca channel blockers or prazosin

Treatment of the primary form involves avoidance of cold, smoking cessation, and, if stress is a triggering factor, relaxation techniques (eg, biofeedback) or counseling. Drugs are used more often than behavioral treatments because of convenience. Vasodilating Ca channel blockers (extended-release nifedipine 60 to 90 mg po once/day, amlodipine 5 to 20 mg po once/day, felodipine 2.5 to 10 mg po bid, or isradipine 2.5 to 5 mg po bid) are most effective, followed by prazosin 1 to 5 mg po once/day or bid. Topical nitroglycerine paste, pentoxifylline 400 mg po bid or tid with meals, or both may be effective, but no evidence supports routine use. β-Blockers, clonidine, and ergot preparations are contraindicated because they cause vasoconstriction and may trigger or worsen symptoms.

Treatment of the secondary form focuses on the underlying disorder. Ca channel blockers or prazosin is also indicated. Antibiotics, analgesics, and, occasionally, surgical debridement may be necessary for ischemic ulcers. Low-dose aspirin may prevent thrombosis but theoretically may worsen vasospasm via prostaglandin inhibition. IV prostaglandins (alprostadil, epoprostenol, iloprost) appear to be effective and may be an option for patients with ischemic digits. However, these drugs are not yet widely available, and their role is yet to be defined. Cervical or local sympathectomy is controversial; it is reserved for patients with progressive disability unresponsive to all other measures, including treatment of underlying disorders. Sympathectomy often abolishes the symptoms, but relief may last only 1 to 2 yr.

Last full review/revision January 2008 by John W. Hallett, Jr., MD