What
Is Raynaud's Phenomenon?
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.
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What
Happens During an Attack?
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.
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How
Is Raynaud's Phenomenon Classified?
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.
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How
Does a Doctor Diagnose Raynaud's Phenomenon?
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. (See the box for the criteria doctors use to diagnose primary or secondary
Raynaud's phenomenon.)
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.
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Diagnostic
Criteria for Raynaud's Phenomenon
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
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What
Is the Treatment for Raynaud's
Phenomenon?
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.
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Self-Help Reminders
- Take action during an attack
- Keep warm
- Don't smoke
- Control stress
- Exercise regularly
- See a doctor if questions
or concerns develop
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