Raynaud's Phenomenon
On this page:
Raynaud's phenomenon is a disorder that affects the blood
vessels in the fingers, toes, ears, and nose. This disorder is
characterized by episodic attacks, called vasospastic attacks, that
cause the blood vessels in the digits (fingers and toes) to constrict
(narrow). Raynaud's phenomenon can occur on its own, or it can be
secondary to another condition such as scleroderma or lupus.
Although estimates vary, recent surveys show that
Raynaud's phenomenon may affect 5 to 10 percent of the general
population in the United States. Women are more likely than men to have
the disorder. Raynaud's phenomenon appears to be more common in people
who live in colder climates. However, people with the disorder who live
in milder climates may have more attacks during periods of colder
weather.
Return to top 
For most people, an attack is usually triggered by
exposure to cold or emotional stress. In general, attacks affect the
fingers or toes but may affect the nose, lips, or ear lobes.
Reduced Blood Supply to the Extremities
When a person is exposed to cold, the body's normal
response is to slow the loss of heat and preserve its core temperature.
To maintain this temperature, the blood vessels that control blood flow
to the skin surface move blood from arteries near the surface to veins
deeper in the body. For people who have Raynaud's phenomenon, this
normal body response is intensified by the sudden spasmodic contractions
of the small blood vessels (arterioles) that supply blood to the fingers
and toes. The arteries of the fingers and toes may also collapse. As a
result, the blood supply to the extremities is greatly decreased,
causing a reaction that includes skin discoloration and other
changes.
Changes in Skin Color and Sensation
Once the attack begins, a person may experience three
phases of skin color changes (white, blue, and red) in the fingers or
toes. The order of the changes of color is not the same for all people,
and not everyone has all three colors. Pallor (whiteness) may occur in
response to spasm of the arterioles and the resulting collapse of the
digital arteries. Cyanosis (blueness) may appear because the fingers or
toes are not getting enough oxygen-rich blood. The fingers or toes may
also feel cold and numb. Finally, as the arterioles dilate (relax) and
blood returns to the digits, rubor (redness) may occur. As the attack
ends, throbbing and tingling may occur in the fingers and toes. An
attack can last from less than a minute to several hours.
Return to top
Doctors classify Raynaud's phenomenon as either the
primary or the secondary form. In medical literature, "primary Raynaud's
phenomenon" may also be called Raynaud's disease, idiopathic Raynaud's
phenomenon, or primary Raynaud's syndrome. The terms idiopathic and
primary both mean that the cause is unknown.
Primary Raynaud's Phenomenon
Most people who have Raynaud's phenomenon have the primary
form (the milder version). A person who has primary Raynaud's phenomenon
has no underlying disease or associated medical problems. More women
than men are affected, and approximately 75 percent of all cases are
diagnosed in women who are between 15 and 40 years old.
People who have only vasospastic attacks for several
years, without involvement of other body systems or organs, rarely have
or will develop a secondary disease (that is, a connective tissue
disorder such as scleroderma) later. Several researchers who studied
people who appeared to have primary Raynaud's phenomenon over long
periods of time found that less than 9 percent of these people developed
a secondary disease.
Secondary Raynaud's Phenomenon
Although secondary Raynaud's phenomenon is less common
than the primary form, it is often a more complex and serious disorder.
Secondary means that patients have an underlying disease or condition
that causes Raynaud's phenomenon. Connective tissue diseases are the
most common cause of secondary Raynaud's phenomenon. Some of these
diseases reduce blood flow to the digits by causing blood vessel walls
to thicken and the vessels to constrict too easily. Raynaud's phenomenon
is seen in approximately 85 to 95 percent of patients with scleroderma
and mixed connective tissue disease, and it is present in about
one-third of patients with systemic lupus erythematosus. Raynaud's
phenomenon also can occur in patients who have other connective tissue
diseases, including Sjögren's syndrome, dermatomyositis, and
polymyositis.
Possible causes of secondary Raynaud's phenomenon, other
than connective tissue diseases, are carpal tunnel syndrome and
obstructive arterial disease (blood vessel disease). Some drugs,
including beta-blockers (used to treat high blood pressure), ergotamine
preparations (used for migraine headaches), certain agents used in
cancer chemotherapy, and drugs that cause vasoconstriction (such as some
over-the-counter cold medications and narcotics), are linked to
Raynaud's phenomenon.
People in certain occupations may be more vulnerable to
secondary Raynaud's phenomenon. Some workers in the plastics industry
(who are exposed to vinyl chloride) develop a scleroderma-like illness,
of which Raynaud's phenomenon can be a part. Workers who operate
vibrating tools can develop a type of Raynaud's phenomenon called
vibration-induced white finger.
People with secondary Raynaud's phenomenon often
experience associated medical problems. The more serious problems are
skin ulcers (sores) or gangrene (tissue death) in the fingers or toes.
Painful ulcers and gangrene are fairly common and can be difficult to
treat. In addition, a person may experience heartburn or difficulty in
swallowing. These two problems are caused by weakness in the muscle of
the esophagus (the tube that takes food and liquids from the mouth to
the stomach) that can occur in people with connective tissue
diseases.
Return to top
If a doctor suspects Raynaud's phenomenon, he or she will ask
the patient for a detailed medical history. The doctor will then examine the
patient to rule out other medical problems. The patient might have a vasospastic
attack during the office visit, which makes it easier for the doctor to diagnose
Raynaud's phenomenon. Most doctors find it fairly easy to diagnose Raynaud's
phenomenon but more difficult to identify the form of the disorder.
Nailfold capillaroscopy (study of capillaries under a
microscope) can help the doctor distinguish between primary and
secondary Raynaud's phenomenon. During this test, the doctor puts a drop
of oil on the patient's nailfolds, the skin at the base of the
fingernail. The doctor then examines the nailfolds under a microscope to
look for abnormalities of the tiny blood vessels called capillaries. If
the capillaries are enlarged or deformed, the patient may have a
connective tissue disease.
The doctor may also order two particular blood tests, an
antinuclear antibody test (ANA) and an erythrocyte sedimentation rate
(ESR). The ANA test determines whether the body is producing special
proteins (antibodies) often found in people who have connective tissue
diseases or other autoimmune disorders. The ESR test is a measure of
inflammation in the body and tests how fast red blood cells settle out
of unclotted blood. Inflammation in the body causes an elevated
ESR.
|
Primary Raynaud's Phenomenon
- Periodic vasospastic attacks of pallor or cyanosis (some
doctors include the additional criterion of the presence of
these attacks for at least 2 years)
- Normal nailfold capillary pattern
- Negative antinuclear antibody test
- Normal erythrocyte sedimentation rate
- Absence of pitting scars or ulcers of the skin, or gangrene
(tissue death) in the fingers or toes
Secondary Raynaud's Phenomenon
- Periodic vasospastic attacks of pallor and cyanosis
- Abnormal nailfold capillary pattern
- Positive antinuclear antibody test
- Abnormal erythrocyte sedimentation rate
- Presence of pitting scars or ulcers of the skin, or gangrene
in the fingers or toes
|
Return to top
The aims of treatment are to reduce the number and
severity of attacks and to prevent tissue damage and loss in the fingers
and toes. Most doctors are conservative in treating patients with
primary and secondary Raynaud's phenomenon; that is, they recommend
nondrug treatments and self-help measures first. Doctors may prescribe
medications for some patients, usually those with secondary Raynaud's
phenomenon. In addition, patients are treated for any underlying disease
or condition that causes secondary Raynaud's phenomenon.
Nondrug Treatments and Self-Help Measures
Several nondrug treatments and self-help measures can
decrease the severity of Raynaud's attacks and promote overall
well-being.
- Take action during an attack--An attack should not be
ignored. Its length and severity can be lessened by a few simple
actions. The first and most important action is to warm the hands or
feet. In cold weather, people should go indoors. Running warm water
over the fingers or toes or soaking them in a bowl of warm water will
warm them. Taking time to relax will further help to end the attack.
If a stressful situation triggers the attack, a person can help stop
the attack by getting out of the stressful situation and relaxing.
People who are trained in biofeedback can use this technique along
with warming the hands or feet in water to help lessen the attack.
- Keep warm--It is important not only to keep the extremities
warm but also to avoid chilling any part of the body. In cold weather,
people with Raynaud's phenomenon must pay particular attention to
dressing. Several layers of loose clothing, socks, hats, and gloves or
mittens are recommended. A hat is important because a great deal of
body heat is lost through the scalp. Feet should be kept dry and warm.
Some people find it helpful to wear mittens and socks to bed during
winter. Chemical warmers, such as small heating pouches that can be
placed in pockets, mittens, boots, or shoes, can give added protection
during long periods outdoors. People who have secondary Raynaud's
phenomenon should talk to their doctors before exercising outdoors in
cold weather.
People with Raynaud's phenomenon should also be
aware that air conditioning can trigger attacks. Turning down the air
conditioning or wearing a sweater may help prevent attacks. Some
people find it helpful to use insulated drinking glasses and to put on
gloves before handling frozen or refrigerated foods.
- Quit smoking--The nicotine in cigarettes causes the skin
temperature to drop, which may lead to an attack.
- Control stress--Because stress and emotional upsets may
trigger an attack, particularly for people who have primary Raynaud's
phenomenon, learning to recognize and avoid stressful situations may
help control the number of attacks. Many people have found that
relaxation or biofeedback training can help decrease the number and
severity of attacks. Biofeedback training teaches people to bring the
temperature of their fingers under voluntary control. Local hospitals
and other community organizations, such as schools, often offer
programs in stress management.
- Exercise--Many doctors encourage patients who have
Raynaud's phenomenon, particularly the primary form, to exercise
regularly. Most people find that exercise promotes overall well-being,
increases energy level, helps control weight, and promotes restful
sleep. Patients with Raynaud's phenomenon should talk to their doctors
before starting an exercise program.
- See a doctor--People with Raynaud's phenomenon should see
their doctors if they are worried or frightened about attacks or if
they have questions about caring for themselves. They should always
see their doctors if attacks occur only on one side of the body (one
hand or one foot) and any time an attack results in sores or ulcers on
the fingers or toes.
Treatment With Medications
People with secondary Raynaud's phenomenon are more likely
than those with the primary form to be treated with medications. Many
doctors believe that the most effective and safest drugs are
calcium-channel blockers, which relax smooth muscle and dilate the small
blood vessels. These drugs decrease the frequency and severity of
attacks in about two-thirds of patients who have primary and secondary
Raynaud's phenomenon. These drugs also can help heal skin ulcers on the
fingers or toes.
Other patients have found relief with drugs called alpha
blockers that counteract the actions of norepinephrine, a hormone that
constricts blood vessels. Some doctors prescribe a nonspecific
vasodilator (drug that relaxes blood vessels), such as nitroglycerine
paste, which is applied to the fingers, to help heal skin ulcers.
Patients should keep in mind that the treatment for Raynaud's phenomenon
is not always successful. Often, patients with the secondary form will
not respond as well to treatment as those with the primary form of the
disorder.
Patients may find that one drug works better than another.
Some people may experience side effects that require stopping the
medication. For other people, a drug may become less effective over
time. Women of childbearing age should know that the medications used to
treat Raynaud's phenomenon may affect the growing fetus. Therefore,
women who are pregnant or are trying to become pregnant should avoid
taking these medications if possible.
- Take action during an attack
- Keep warm
- Don't smoke
- Control stress
- Exercise regularly
- See a doctor if questions or concerns develop
|
Return to top
Researchers are studying ways to better diagnose Raynaud's
phenomenon and predict and monitor its course and association with other
diseases. They are also evaluating the use of new drugs to improve blood
flow in Raynaud's phenomenon; for example, the prostaglandins iloprost
and alprostadil, the high blood pressure drug losartan, and a substance
applied to the skin that generates the blood vessel dilating gas nitric
oxide. Basic investigators are studying the molecular mechanisms behind
Raynaud's phenomenon, the anatomy of blood vessels, and possible genetic
associations. Some researchers are studying the use of biofeedback to
control attacks. Researchers in scleroderma and other connective tissue
diseases are also investigating Raynaud's phenomenon in relation to
these diseases.
Return to top
National Institute
of Arthritis and Musculoskeletal and
Skin Diseases Information Clearinghouse
National Heart, Lung, and
Blood Institute Information Center
American College of Rheumatology
Arthritis Foundation
Scleroderma Foundation
Return to top
