RAYNAUD'S PHENOMENON
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). 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.
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.
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
much 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. For most people with lupus, Raynaud's
phenomenon acts like the primary form of the disorder. 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. In addition, people
whose fingers are subject to repeated stress, such as typing
or playing the piano, are more vulnerable to the disorder.
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.
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 below 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.
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
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.
-
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.
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
What Research Is
Being Conducted To Help People Who Have Raynaud's Phenomenon?
Researchers are
studying the use of other drugs to treat Raynaud's phenomenon;
for example, oral and intravenous prostaglandins, such as iloprost.
Other investigators are studying the molecular mechanisms behind
Raynaud's phenomenon and the anatomy of blood vessels. Several
medical centers in the United States are studying the use of
biofeedback to control attacks. Researchers studying scleroderma
and other connective tissue diseases are also investigating
Raynaud's phenomenon in relation to these diseases.
Where Can People
Get More Information About Raynaud's Phenomenon?
-
- Arthritis Foundation
1330 West Peachtree Street
Atlanta, GA 30309
404/872-7100 or call your local chapter (listed in the telephone
book)
800/283-7800
World Wide Web address: http://www.arthritis.org
This is the main
voluntary organization devoted to all forms of arthritis. The
Foundation publishes a free pamphlet on Raynaud's phenomenon
and also provides physician referrals.
The NIAMS gratefully
acknowledges the assistance of Phillip J. Clements, M.D., of
the University of California, Los Angeles; Jay D. Coffman, M.D.,
of the Boston University Medical Center; and Frederick M. Wigley,
M.D., of The Johns Hopkins University School of Medicine in
the preparation and review of this fact sheet.
The National Arthritis
and Musculoskeletal and Skin Diseases Information Clearinghouse
(NAMSIC) is a public service sponsored by the NIAMS that provides
health information and information sources. The NIAMS, a part
of the National Institutes of Health (NIH), leads the Federal
medical research effort in arthritis and musculoskeletal and
skin diseases. The NIAMS sponsors research and research training
throughout the United States as well as on the NIH campus in
Bethesda, MD, and disseminates health and research information.
AMT 6/96, Updated
1/97
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