Patient Information Raynaud Ph

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Patient information: Raynaud phenomenon

Last literature review version 18.3: settembre 2010 | This topic last updated: luglio 6, 2007

OVERVIEW The Raynaud phenomenon (RP) is a condition in which some of the body's blood vessels

(most commonly those in the fingers and toes) constrict in an exaggerated way in response to cold or

emotional stress.

Normally, blood vessels supplying the skin constrict or narrow in response to cold temperatures. This

reaction, called "vasoconstriction," decreases blood flow to the skin, which helps to minimize heat loss and

preserve a normal internal or "core" temperature. In warm temperatures these same blood vessels dilate,

allowing heat to leave the body.

In people with Raynaud phenomenon (RP), the mechanisms that control vasoconstriction are thought to

be altered or defective. The vessels constrict in an exaggerated way in response to cold and emotional or

physical stress. The severe constriction restricts blood flow to the affected areas and causes the skin to

change to a white color, when there is virtually no blood flow. The skin may then become a purplish-blue

color (called acrocyanosis), caused by low oxygen levels in the blood. When the vessel recovers, it dilates,

allowing blood flow to resume; the skin may become very pink or red.

RAYNAUD PHENOMENON RISK FACTORS It is estimated that RP affects 3 to 5 percent of the

general population.

Primary disease Patients who have Raynaud phenomenon without a related disease are said to have

primary RP. The underlying reason for blood vessel sensitivity to cold is uncertain. Primary RP may be a

family trait, suggesting that one or more genes may be responsible.

Primary RP accounts for the majority of case, and is more common among women, younger age groups,

and in family members of patients with RP. Fortunately, most patients with primary RP are not

significantly disabled by the condition and respond well to treatment.

Secondary disease Some patients with secondary RP have an illness, such as scleroderma or

systemic lupus erythematosus, that injures or alters the blood vessels and affects how they react to

stimuli such as cold and stress. Secondary RP can be more difficult to manage because it is linked to an

underlying condition that can physically damage the blood vessel. (See "Patient information: Systemic

lupus erythematosus (SLE)".)

RAYNAUD PHENOMENON CAUSES The normal control of blood vessel responses to cold and other

stimuli is complex, involving the central nervous system, peripheral sensory nerves, and molecules

released by circulating cells or from the inner lining of the blood vessel itself (call the endothelium).

Alpha adrenergic receptors are proteins on the surface of the muscle cells of blood vessels. They help to

maintain vascular "tone". When stimulated, the receptors trigger blood vessel to constrict in response tocertain stimuli such as stress or cold. Receptors that are overly sensitive to adrenalin-like chemicals,

released from nerves in the skin, could be responsible for the exaggerated responses to cold that people

with RP experience.

An RP attack can be triggered by exposure to cold temperature or even by a shift in temperature from

Official reprint from UpToDatewww.uptodate.com

2011 UpToDate

AuthorFredrick M Wigley, MD

Section EditorJohn S Axford, DSc, MD, FRCP

Deputy EditorJerry M Greene, MD

Pagina 1 di 6aynaud phenomenon

28/02/2011ttp://www.uptodate.com/contents/patient-information-raynaud-phenomenon?view=print


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warm to cool. As a result, even mildly cold exposures, such as that caused by air-conditioning or by the

cold of the refrigerated food section in a grocery store, can cause a RP attack. Experiencing a general

body chill can also trigger an attack, even if the hands and feet are kept warm. Emotional stress and

being startled can cause an attack of RP due to the release of nerve transmitter substances; these

substances activate the alpha receptors, which signal the blood vessels to narrow.

RAYNAUD PHENOMENON SYMPTOMS Most often, an RP attack affects the blood vessels in the

fingers. In a typical RP attack, the fingers (or toes) become suddenly cold as the blood vessels constrict.

The skin color changes markedly, and may become pale (called a "white attack") or a purple or blue color

(called a "blue attack"). Usually, an attack of RP begins in a single finger and then spreads to other fingersin both hands. The index, middle, and ring fingers are most commonly involved, while the thumb is often

not affected (picture 1).

Patients may experience discomfort, including a "pins and needles" feeling, aching, numbness, or

clumsiness of the affected hand(s). The feeling of true pain occurs in secondary RP and is caused by a

critical and prolonged loss of blood flow to the tissues.

Blood vessels supplying the skin of the ears, nose, face, knees, and nipples can also be affected and the

skin in these areas may become pale or bluish in color after cold exposure. Mottling (a bluish

discoloration) of the skin of the arms and legs might also appear. Attacks affecting the toes are also

common, although patients tend to complain of these less frequently.Symptoms of RP resolve as the provoking factor (cold or stress) is removed. After leaving the cold area

and rewarming, the discoloration resolves after 15 to 20 minutes and, as normal blood flow resumes, the

skin "blushes" or becomes pink.

Patients with severe secondary RP can sometimes experience a serious decrease in blood flow that does

not resolve even after the provoking factor or cold is removed. Pain and ulceration of the skin (usually on

the tips of the fingers and toes) can result.

RAYNAUD PHENOMENON DIAGNOSIS Raynaud phenomenon is diagnosed based on the patient's

description of a typical attack following cold exposure. By asking questions, a healthcare provider can

usually tell if Raynaud phenomenon or another more common condition is causing cold hands or feet.

RAYNAUD PHENOMENON TREATMENT By taking some simple steps, you can reduce the frequency

of RP attacks. A medicine might also be used to help control the symptoms.

Avoid sudden cold exposure Use strategies to keep the whole body warm and avoid rapidly shifting

temperature, cold breezes, and damp cold conditions. These include dressing warmly, wearing layer

clothing such as thermal underwear, donning a hat, and using mittens or gloves.

Help end an attack Methods include placing the hands under warm water or in a warm place (such

as in the armpits), or rotating the arms in a whirling windmill pattern.

Avoid smoking The nicotine and other chemicals in cigarettes cause the blood vessels to constrictand can aggravate RP. (See "Patient information: Quitting smoking".)

Avoid medications that cause vasoconstriction Such medications include decongestants containing

phenylephrine or pseudoephedrine, other amphetamines, diet pills, some migraine remedies containing

ergotamine, and herbs containing ephedra.

Reduce stress and use relaxation techniques to reduce anxiety.

Medicine If the measures above are not sufficient, your doctor or nurse might recommend a medicine.

Medicines called calcium channel blockers are the most commonly used and can reduce both the

frequency and severity of RP attacks. You might need to take the medicine when you are exposed to coldtemperatures, such as during the winter months.

Secondary Raynaud phenomenon More aggressive treatment is sometimes needed for people with

secondary Raynaud phenomenon. Most people need a medicine to reduce the frequency of attacks and

prevent injury to the skin on the fingers and toes.




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Several medicines have been studied for people who do not respond to calcium channel blockers. These

include nitrates (topical nitroglycerin), blood pressure medications (prazosin or an angiotensin receptor

inhibitors like losartan), phosphodiesterase inhibitors (sildenafil, pentoxifylline or cilostazol), endothelin-1

inhibitors (bosentan), antidepressant medication (fluoxetine), or prostaglandins (prostacyclin).

Hospitalization may be required if an attack of RP does not resolve and blood flow to a finger or toe is

seriously restricted. Treatment in this situation requires additional medications to dilate the blood vessels

and to prevent blood clots. In some cases, medication or surgery may be used to block the nerves that

trigger vasoconstriction in the affected area.

In rare instances, treatment may be ineffective in reversing the vasoconstriction. Ulceration of the skin

may result. If all other treatments have failed, surgical amputation of the affected finger or toe may

become necessary.

WHERE TO GET MORE INFORMATION Your healthcare provider is the best source of information for

questions and concerns related to your medical problem.

This article will be updated as needed every four months on our web site (www.uptodate.com/patients).

Related topics for patients, as well as selected articles written for healthcare professionals, are also

available. Some of the most relevant are listed below.

Patient level information

Patient information: Systemic lupus erythematosus (SLE)

Patient information: Quitting smoking

Professional level information

Clinical manifestations and diagnosis of the Raynaud phenomenon

Nonpharmacologic therapy for the Raynaud phenomenon

Pathogenesis of the Raynaud phenomenon

Pharmacologic and surgical treatment of the Raynaud phenomenon

The following organizations also provide reliable health information.

The National Institutes of Health (NIH)

(www.nlm.nih.gov/medlineplus/healthtopics.html)

The National Institute of Arthritis and Musculoskeletal and Skin Diseases

(www.niams.nih.gov/hi)

The Arthritis Foundation

(www.arthritis.org/conditions/DiseaseCenter/raynauds.asp)

The Arthritis Society

(www.arthritis.ca)

American College of Rheumatology

(www.rheumatology.org)

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REFERENCES




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1. Wigley, FM, Flavahan, NA. Raynaud's phenomenon. Rheum Dis Clin North Am 1996; 22:765.

2. Block, JA, Sequeira, W. Raynaud's phenomenon. Lancet 2001; 357:2042.

3. Wigley, FM. Clinical practice. Raynaud's Phenomenon. N Engl J Med 2002; 347:1001.

4. Flavahan, NA, Flavahan, S, Mitra, S, Chotani, MA. The vasculopathy of Raynaud's phenomenon and

scleroderma. Rheum Dis Clin North Am 2003; 29:275.

5. Boin, F, Wigley, FM. Understanding, assessing and treating Raynaud's phenomenon. Curr Opin

Rheumatol 2005; 17:752.

6. Herrick, AL. Pathogenesis of Raynaud's phenomenon. Rheumatology (Oxford) 2005; 44:587.




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GRAPHICS

Raynaud phenomenon

In picture A, the tips of two fingers have turned white. In picture

B, the tips of three fingers have turned purple-blue. Reproducedwith permission from: Wigley, FM. Raynaud's phenomenon. N Engl J Med

2002; 347:1001. Copyright 2002 Massachusetts Medical Society.



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