Raynaud Phenomenon

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Definition

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The Raynaud phenomenon (RP) is an exaggerated vascular response to cold temperature or emotional stress. The phenomenon is manifested clinically by sharply demarcated color changes of the skin of the digits.

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PREVALENCE

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ranged from 3 to 20 percent in women and 3 to 14 percent in men.

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capillaroscopy

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capillaroscopy
Findings on nailfold capillaroscopy in normals and in scleroderma (25x magnification). Top panel: Normal capillary pattern with parallel, evenly spaced, thin capillary loops. (25x magnification). Middle panel: Abnormal capillaroscopy in a patient with scleroderma. The few capillaries are dilated and irregularly distributed. Much of the proximal nail fold has lost its normal complement of capillaries, leaving large, white avascular areas. Bottom panel: More advanced changes in scleroderma. The few remaining capillaries have taken on a bushy configuration, and the proximal nailfold again has large avascular areas. The bushy capillary configuration is more common in dermatomyositis). Reproduced with permission from Richard D Sontheimer, DM. In: Resource Materials in Rheumatology, number 19: Nail changes in rheumatic diseases.

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Nailfold capillaroscopy

Findings on nailfold capillaroscopy in normals and in scleroderma (25x magnification). Top panel: Normal capillary pattern with parallel, evenly spaced, thin capillary loops. (25x magnification). Middle panel: Abnormal capillaroscopy in a patient with scleroderma. The few capillaries are dilated and irregularly distributed. Much of the proximal nail fold has lost its normal complement of capillaries, leaving large, white avascular areas. Bottom panel: More advanced changes in scleroderma. The few remaining capillaries have taken on a bushy configuration, and the proximal nailfold again has large avascular areas. The bushy capillary configuration is more common in dermatomyositis). Reproduced with permission from Richard D Sontheimer, DM. In: Resource Materials in Rheumatology, number 19: Nail changes in rheumatic diseases.

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CLINICAL MANIFESTATIONS

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most often affects the hand; attacks also frequently occur in the toes, but patients are less likely to complain of lower extremity symptoms.

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A typical episode is characterized by

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sudden onset of cold fingers (or toes) in association with sharply demarcated color changes of skin pallor (white attack) and/or cyanotic skin (blue attack)

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With rewarming, the ischemic phase (white or blue attack) usually lasts for

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15 to 20 minutes.

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typically begins in

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a single finger and then spreads to other digits symmetrically in both hands.

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the most frequently involved digits are

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The index, middle, and ring finger
the thumb is often spared entirely

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Clinical criteria

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Definite RP — Repeated episodes of biphasic color changes upon exposure to cold
Possible RP — Uniphasic color changes plus numbness or paresthesia upon exposure to cold
No RP — No color changes upon exposure to cold

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Primary RP

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patients without a definable cause for their vascular events. In this setting, RP is considered to be an exaggeration of normal vasoconstriction to cold exposure. (See "Pathogenesis of the Raynaud phenomenon"). Most investigators feel the term "disease" is inappropriate, and prefer using the term primary RP for otherwise healthy individuals.

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Secondary RP

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Secondary RP refers to those patients with RP in whom an associated disease or cause may underlie the attacks [17]. Other investigators prefer the term Raynaud syndrome

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Primary RP has an age of onset between ......years of age, is more common in ........., and may occur in ............

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15 and 30 , women , multiple family members

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Clinical clues to suggest secondary RP include

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Later age of onset (greater than 40 years)
Male gender
Painful severe events with tissue sign of ischemia (ulceration)
Asymmetric attacks
RP associated with signs or symptoms of another disease
Abnormal laboratory parameters suggesting vascular disease or an autoimmune disorder
RP associated with ischemic signs or symptoms proximal to the fingers (such as the hand or arm) or toes (foot or limb).

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Secondary RP
The most common associated disorders are

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scleroderma,
systemic lupus erythematosus,
other connective tissue diseases,
occlusive vascular disease,
drug effects,
hematologic abnormalities, and
use of vibrating tools:

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Current criteria for the diagnosis of primary Raynaud phenomenon include the following:

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Symmetric episodic attacks
No evidence of peripheral vascular disease
No tissue gangrene, digital pitting, or tissue injury
Negative nailfold capillary examination
Negative antinuclear antibody test and normal erythrocyte sedimentation rate (ESR)

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Approximately .... percent of patients initially diagnosed with primary disease may transition to secondary RP each year

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one

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T/F The combination of autoantibodies and nailfold capillary microscopy may be more informative than either test alone.

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True

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The following general measures are helpful for all patients with RP

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Avoidance of sudden cold exposure and stress reduction
Use of strategies to keep the whole body warm, including dressing warmly (eg, with thermal underwear and heat conserving hat)
Keeping digits warm (eg, mittens or electric hand warmers instead of gloves).
Knowledge of methods to help terminate an attack of RP. These include placing the hands under warm water or in a warm place (such as the axilla), or rotating arms in a whirling or windmill pattern.
Avoidance of rapidly changing temperatures, such as quickly moving from a hot environment (90 degrees F) into an air-conditioned room (70 degrees F); cool breezes, or humid cold air is also recommended.
Avoidance of smoking is recommended since regular smokers are sensitized to the vasoconstrictive properties of cigarettes; the response in patients with RP does not appear to be different from that in normals [5]. Avoiding second hand smoke is prudent.
Avoidance of sympathomimetic drugs (such as decongestants, amphetamines, diet pills, herbs containing ephedra) is generally recommended, but studies of the true impact of over-the-counter preparations (such as cold medications) have not yet been performed.
Discontinuing caffeine containing beverages has also been recommended, but xanthines transiently reduce peripheral vascular resistance.
Patients with vibration-induced RP should avoid use of vibrating tools.

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BEHAVIORAL THERAPY

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In biofeedback training, patients are comfortably seated with thermistors attached to the fingertips. Patients are then asked by the therapist to alter the temperature in the fingers while readings of the skin temperature are "fed back" to the patient using auditory or visual signals.
Autogenic training teaches the use of relaxation inducing self-statements that suggest warmth and heaviness [14]. This method has been used alone or in combination with temperature biofeedback.

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DRUG THERAPY

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Calcium channel blockers
Other vasodilators
Sympatholytic agents
Prostaglandins

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Classical conditioning

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pairing the unconditioned stimulus of warm water applied to the hands with a conditioned stimulus of exposure of the whole body to cold. After repeated pairings, exposure to cold alone elicits vasodilation in the hands, which is the conditioned response. Using this conditioning procedure, subjects with RP can exhibit significant increases in digital temperature during a cold stress relative to untreated control subjects