CME

Treating Raynaud phenomenon: Beyond staying warm

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Release date: October 1, 2017
Expiration date: September 30, 2018
Estimated time of completion: 1 hour

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ABSTRACT

Raynaud phenomenon is an overactive vascular response to cold and emotional stress that results in cutaneous color changes and sensory symptoms in the digits. It can be idiopathic (primary) or secondary to another condition; the latter can be more severe and more apt to lead to ischemic complications such as digital ulceration and even loss of digits. If nonpharmacologic interventions prove inadequate, then vasodilator agents are used.

KEY POINTS

  • Primary Raynaud phenomenon occurs in the absence of any underlying disease process. Secondary Raynaud phenomenon occurs in concert with another disease, frequently rheumatic.
  • Young patients with mild Raynaud phenomenon, normal nailfold capillaries, and no additional symptoms or signs to suggest a rheumatic or other underlying disease can be followed carefully by the primary care doctor and do not require further serologic workup or referral to a specialist.
  • Nonpharmacologic interventions, ie, cold avoidance and stress management, are first-line for all patients.
  • Calcium channel blockers are first-line drugs and should be titrated to the maximum tolerated dose before adding or switching to other agents.
  • The goal of treatment should not be to eliminate Ray­naud attacks completely but to improve quality of life and prevent ischemic complications.

 

References

Raynaud phenomenon is an overactive vascular response to cold and emotional stress that results in cutaneous color changes and sensory symptoms of the digits (Figure 1). It can occur in isolation as primary Raynaud phenomenon or secondary to another disease process. It is thought to be triggered by a heightened sympathetic vasoconstrictive response of small arteriovenous anastomoses in the fingers, toes, ears, and tip of the nose. These structures play a key role in maintaining a stable core body temperature by cutaneous thermoregulation.1

(A) White digits with intense vasoconstriction in Raynaud phenomenon; (B) blue digits with hypoxemic venous stasis; (C) red digits with hyperemic reperfusion.
Figure 1. (A) White digits with intense vasoconstriction in Raynaud phenomenon; (B) blue digits with hypoxemic venous stasis; (C) red digits with hyperemic reperfusion.

Secondary Raynaud phenomenon can be seen with a wide array of systemic conditions as well as environmental and drug exposures. It is a frequent feature of autoimmune rheumatic conditions such as systemic sclerosis, mixed connective tissue disease, systemic lupus erythematosus, and dermatomyositis. Less commonly, cryoproteinemias, paraneoplastic syndromes, hypothyroidism, and carpal tunnel syndrome can be associated with or cause Raynaud phenomenon. Vibratory trauma (eg, from using a jackhammer) and drugs (eg, vasopressors, stimulants, ergots, chemotherapeutic agents) can also cause Raynaud phenomenon.1

A variety of disorders that cause vasospasm or vascular occlusion of the peripheral circulation can mimic typical Raynaud phenomenon, including peripheral nerve injury,2 complex regional pain syndrome,3 occlusive vascular disease, vasculitis, acrocyanosis,4 and thoracic outlet syndrome.

The prevalence of Raynaud phenomenon is not exactly known, in part due to geographic differences in climate and variation in methods of assessment. However, a 2015 systematic review and meta-analysis of primary Raynaud phenomenon determined a pooled prevalence of 4.85% (95% confidence interval [CI] 2.08%–8.71%) in the general population.5 Accordingly, accurate identification and management of this condition is a useful skill for the internist.

COLD SENSITIVITY AND COLOR CHANGES

Because there are no confirmatory diagnostic tests for this condition, there are no formal diagnostic criteria. However, many experts agree that Raynaud phenomenon can be diagnosed clinically when patients report:

  • Unusual sensitivity of the fingers to cold, manifesting as pain or paresthesia (eg, tingling, pricking, numbness), and
  • Color changes of the fingers when exposed to cold, specifically pale white or blue-black, or both.6

Provocative testing such as submerging patients’ hands in cold water is not recommended, as it is distressing to the patient and inconsistent in triggering an event.

Pain is a symptom of critical digital ischemia.

The skin color changes are due to rapid alterations in blood flow in digital skin. The pale white is due to markedly reduced or absent flow secondary to intense vasoconstriction, the blue-black is due to hypoxemic venous stasis, and the red blush is due to hyperemic reperfusion (Figure 1). However, not all patients have all 3 phases of the classic triphasic color changes, and color changes may not follow a set sequence.

Raynaud phenomenon can also occur in other areas of the body that have thermoregulatory vessels, such as the toes, ears, nipples, tongue, and nose. While some patients with Raynaud phenomenon have a finger that is more sensitive than the others, repeated isolated single-digit or asymmetric events without typical progression to all fingers suggest a secondary local structural disease requiring further investigation (see below).

Symptoms related to Raynaud often mimic sensory changes including paresthesias, numbness, aching, and clumsiness of the hand. Abnormal vascular reactivity has been implicated as a causative factor in several disorders, such as migraine headache, preeclampsia, and variant angina. While case reports, case series, and some controlled studies have linked Raynaud phenomenon and these conditions, there is no solid evidence of a systemic vaso­spastic disorder in patients with primary Raynaud phenomenon.

Raynaud phenomenon is triggered by more than just a cold ambient temperature. Provocation can occur during movement from warmer to relatively cooler temperatures, as well as during episodes of elevated sympathetic activity (eg, emotional distress or fear). In fact, maintaining full body warmth as well as emotional equilibrium are the most important strategies to reduce the frequency of attacks.

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