Raynaud’s Phenomenon/Disease

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Overview

Peripheral vasospasm can occur as an isolated disease entity (Raynaud's disease) or in association with another disease process, such as scleroderma and other rheumatic diseases (Raynaud's phenomenon). Typically, the digits are involved, but, rarely, the nose, ears, nipples, and other exposed areas can be affected. The therapeutic strategy is to minimize the circumstances that trigger peripheral vasospasm and to treat the patient with pharmacologic agents that control the clinical reaction.

First Steps

1. For patients with mild infrequent Raynaud's phenomenon, reassurance and avoidance of cold exposure may control the problem. Such individuals should be advised to dress warmly, and to wear gloves or mittens when cold exposure is anticipated. In addition, smoking is contraindicated.
2. For patients with moderate to severe Raynaud's phenomenon, drug treatment is beneficial. The calcium channel blockers amlodipine 2.5-10 mg once daily, nifedipine 10-30 mg 3 times daily, or diltiazem 30-60 mg four times daily will control symptoms in the majority of patients.

Alternative Steps

1. The phosphodiesterase inhibitor sildenafil 50 mg twice daily is useful in patients who are not adequately treated with calcium channel blockers. In addition, tadalafil has been reported to be useful in at least one patient.
2. Patients who do not respond to these measures may be referred to a specialist in the treatment of Raynaud's. Additional therapeutic alternatives may include prostacyclin infusion, and various approaches to adrenergic blockade including sympathetic surgical denervation.

Ancillary Steps

1. Patients should avoid excess exposure to cold. Warm, loose-fitting clothing is preferred, and the entire body must remain warm. Submerging involved digits in warm water to terminate vasospastic episodes may decrease the occurrence of ischemic damage.
2. Discontinuation of smoking ameliorates Raynaud's phenomenon. Passive inhalation of smoke also may trigger episodes. Even a single cigarette per month may be enough to cause Raynaud's persistence.
3. Trauma should be minimized because of delayed wound-healing and tendency for persistent ulcerations. This may influence choice of, or even dictate changes in, types of occupation.
4. Work-up of Raynaud's phenomenon will reveal an associated systemic disorder in many cases. Therapy of the underlying disease may results in improvement of the associated vasospasm.
5. Debridement, systemic antibiotics, and vapor-permeable membranes may be useful for treating associated ulcerations.

Pitfalls

1. Acral pitting and/or ulcerations may complicate the course of Raynaud's phenomenon in many cases.
2. The associated systemic disease may not surface for years after the initial occurrence of Raynaud's phenomenon. Therefore, work-ups need to be repeated at regular intervals.
3. Certain drugs, such as ergotamines and alpha-blockers, can aggravate or precipitate Raynaud's disease. The possible role of medications in disease activity should be examined carefully.
4. Calcium channel blockers can produce adverse side effects, including hypotension, CNS toxicity, aggravation of esophageal reflux, and peripheral edema

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