Questions and
Answers about Raynaud's Phenomenon
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Publication Date May
2001
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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What Research Is Being
Conducted To Help People Who Have Raynaud's Phenomenon?
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.
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Where Can People Get More
Information About Raynaud's Phenomenon?
National Institute of Arthritis
and Musculoskeletal and
Skin Diseases Information Clearinghouse
National Institutes of Health
1 AMS Circle
Bethesda, MD 20892-3675
Phone: 301-495-4484 or
877-22-NIAMS (226-4267) (free of charge)
TTY: 301-565-2966
Fax: 301-718-6366
http://www.niams.nih.gov/
The clearinghouse provides
information about various forms of arthritis and rheumatic disease
and bone, muscle, and skin diseases. It distributes patient and
professional education materials and refers people to other
sources of information. Additional information and updates can
also be found on the NIAMS Web site.
National Heart, Lung, and Blood
Institute Information Center
National Institutes of Health
P.O. Box 30105
Bethesda, MD 20824-0105
Phone: 301-592-8573
Fax: 301-592-8563
E-mail:
NHLBIinfo@rover.nhlbi.nih.gov
http://www.nhlbi.nih.gov/
The National Heart, Lung, and Blood
Institute (NHLBI) provides leadership for a national program in
diseases of the heart, blood vessels, lung, and blood; blood
resources; and sleep disorders. The NHLBI Information Center
develops and maintains information on numerous topics to respond
to inquiries on specific diseases related to the heart, lungs, and
blood.
American College of Rheumatology
1800 Century Place, Suite 250
Atlanta, GA 30345
Phone: 404-633-3777
E-mail:
acr@rheumatology.org
www.rheumatology.org
This professional organization of
rheumatologists (doctors specializing in arthritis and related
conditions such as Raynaud's phenomenon) and associated health
professionals is involved in research, education, and patient
care. It provides referrals to rheumatologists and other health
professionals.
Arthritis Foundation
1330 West Peachtree Street
Atlanta, GA 30309
Phone: 404-872-7100 or 800-283-7800 (free of charge)
or call your local chapter (listed in the telephone directory)
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.
Lupus Foundation of America,
Inc.
2000 L Street, N.W., Suite 710
Washington, DC 20036
Phone: 202-349-1155
Toll-Free: (800)558-0121
Fax: 202-349-1156
E-mail:
lupusinfo@lupus.org
www.lupus.org
This is the main voluntary
organization devoted to lupus. The LFA offers information and
referral services, health fairs, newsletters, publications,
seminars, support group meetings, hospital visits, and telephone
help lines.
National Sjögren's Syndrome
Association
5815 North Black Canyon Highway, Suite 103
Phoenix, AZ 85015
Phone: 602-433-9844 or 800-395-6772 (free of charge)
Fax: 602-516-0111
E-mail:
NSSA@aol.com
This association promotes public
awareness of Sjögren's syndrome and encourages research into the
cause and cure of the disorder. It also conducts educational and
research programs.
Scleroderma Foundation
12 Kent Way, Suite 101
Byfield, MA 01922
Phone: 800-722-HOPE (4673) (free of charge)
E-mail:
sfinfo@scleroderma.org
www.scleroderma.org
This voluntary organization
publishes information on scleroderma and funds research. It also
offers patient education seminars, support groups, physician
referrals, and information hotlines.
Sjögren's Syndrome Foundation
366 North Broadway
Jericho, NY 11753
Phone: 516-933-6365 or 800-475-6473 (free of charge)
Fax: 516-933-6368
E-mail:
ssf@sjogrens.org
www.sjogrens.org
This organization is devoted to
Sjögren's syndrome. It publishes free pamphlets and a newsletter
for members that provides up-to-date information. It provides
clinic and physician referrals.
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Acknowledgments
The NIAMS gratefully acknowledges
the assistance of Paul Plotz, M.D., NIAMS, NIH; 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 booklet.
The mission of the National
Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS),
a part of the National Institutes of Health (NIH), is to support
research into the causes, treatment, and prevention of arthritis
and musculoskeletal and skin diseases, the training of basic and
clinical scientists to carry out this research, and the
dissemination of information on research progress in these
diseases. The National Institute of Arthritis and Musculoskeletal
and Skin Diseases Information Clearinghouse is a public service
sponsored by the NIAMS that provides health information and
information sources. Additional information can be found on the
NIAMS Web site at
http://www.niams.nih.gov/.
NIH Publication No. 01-4911
** The APSFA has added this
organization to the list. It is not included in the NIAMS
brochure.
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