What Is Raynaud's Disease?
Raynaud's disease and Raynaud's phenomenon are rare
disorders that affect blood vessels. These disorders are marked by brief
episodes of vasospasm (narrowing of the blood vessels). Vasospasm causes
decreased blood flow to the fingers and toes, and rarely to the nose, ears,
nipples, and lips. The fingers are the most commonly affected area, but the
toes also are affected in 40 percent of people with Raynaud's.
When this disorder occurs without any known cause,
it is called Raynaud's disease, or primary Raynaud's. When the condition occurs
along with a likely cause, it is known as Raynaud's phenomenon, or secondary
Raynaud's. Primary Raynaud's is more common and tends to be less severe than
secondary Raynaud's.
When you have primary or secondary Raynaud's, cold
temperatures or stressful emotions can trigger attacks. During these attacks,
there is a brief lack of blood flow to the affected body part(s), and the skin
can temporarily become white then bluish. As blood flow returns to the area,
the skin turns red. The affected areas can throb or feel numb and tingly. With
severe Raynaud's, prolonged or repeated episodes can cause sores or tissue
death (gangrene).
Figure A shows the normal digital
arteries with normal blood flow to the fingers. The inset images show
cross-sections of a normal artery. Figure B shows white discoloration of the
fingertips caused by blocked blood flow. Figure C shows narrowed digital
arteries, causing blocked blood flow and purple discoloration of the
fingertips. The inset images show cross-sections of a narrowed artery blocking
the flow of blood.
It is normal for the body to keep its vital inner
organs warm by limiting blood flow to the arms, legs, fingers, and toes. The
body naturally does this in response to a long period of cold. This response
can cause frostbite. In people with Raynaud's, the response to cold is quicker
and stronger. The response can be triggered by mild or short-lived changes in
temperature, such as:
- Taking something out of the freezer
- Temperatures that dip below 60 degrees
Fahrenheit
In people with Raynaud's, blood flow is more
strongly reduced in response to cold temperatures than in people without the
disorder. When Raynaud's is severe (which is uncommon), exposure to cold for as
little as 20 minutes can cause major tissue damage.
The blood vessels of people with Raynaud's also
physically overreact to stressful emotions. It is normal during times of
psychological stress for the body to release hormones that narrow its blood
vessels. But for people with Raynaud's, this squeezing of blood vessels is
stronger. This results in less blood reaching fingers, toes, and sometimes
other extremities.
Outlook
For most people, primary Raynaud's is more of a
bother than a serious illness and it can usually be managed with minor
lifestyle changes. Secondary Raynaud's can be more difficult to manage, but
several treatments may help prevent or relieve symptoms. Among the most
important treatments for secondary Raynaud's is treating of the underlying
condition.
What Causes Raynaud's Disease?
In most cases of Raynaud's, no cause can be found.
When this happens, the disorder is called primary Raynaud's. When a cause can
be found, the disorder is called secondary Raynaud's. Secondary Raynaud's can
be linked to many different medical or workplace conditions, such as:
- Diseases that damage blood vessels or the nerves
that control the function of blood vessels in the hands and feet
- Repetitive actions that damage the nerves that
control blood vessels in the hands and feet
- Exposure to certain chemicals
- Use of medicines that narrow blood vessels or
affect blood pressure
Diseases
Secondary Raynaud's is especially common in people
who have
scleroderma or
systemic lupus erythematosus. About 9 out of 10 people with
scleroderma have Raynaud's. About 1 out of 3 people with lupus have Raynaud's.
The disease also is linked to other diseases that damage blood vessels or
nerves, including:
Raynaud's also can be associated with thyroid
problems and pulmonary hypertension (high blood pressure in the arteries of the
lungs).
Repetitive Actions
Typing, playing the piano, or another repetitive
action done for long periods of time is often linked to secondary Raynaud's.
Using vibrating tools at work also can make you more likely to develop the
disorder.
Chemicals
Exposure to certain chemicals at work can cause a
scleroderma-like illness that is linked to Raynaud's. Nicotine in cigarette
smoke also can make you more prone to developing Raynaud's.
Medicines
Several medicines are linked to secondary Raynaud's,
including:
- Migraine headache medicines that contain
ergotamine
- Certain cancer medicines
- Some over-the-counter cold or allergy
remedies
- Some blood pressure medicines (beta
blockers)
Injuries to the hands or feet from surgery,
frostbite, or other causes also can lead to secondary Raynaud's.
Who Is At Risk for Raynaud's Disease?
About 3 out of 4 cases of primary Raynaud's occur in
women between the ages of 15 and 40 years, although the reason is unknown.
People in colder climates are also more likely to develop Raynaud's than people
in warmer areas.
What Are the Signs and Symptoms of Raynaud's
Disease?
People with Raynaud's (primary or secondary) have
attacks in response to cold or emotional stress. The attacks can affect the
fingers and toes, and rarely the nose, ears, nipples, or lips. The affected
body parts will usually have two or more of the following changes:
- Look pale due to lack of blood flow
- Look bluish due to a lack of oxygen
- Feel numb, cold, or painful
- Redden and throb or tingle as blood returns to
the affected area
Attacks usually last about 15 minutes. They can last
less than a minute or as long as several hours. Attacks can occur daily or
weekly.
Sometimes attacks affect only one or two fingers or
toes. Different areas may be affected at different times. Attacks can cause
sores or tissue death (gangrene) in people with severe secondary Raynaud's.
However, severe Raynaud's is very uncommon.
How Is Raynaud's Disease Diagnosed?
Raynaud's is usually diagnosed based on a patient's
history of experiencing color changes in their extremities in response to
exposure to cold or emotional stress. A cold simulation test also may be used
to provoke symptoms for the doctor to see.
Doctors use the patient's history of symptoms, a
physical exam, and diagnostic tests to rule out other conditions that might act
like Raynaud's.
Specialists Involved
A rheumatologist (roo-ma-TOL-o-jist) is a doctor who
specializes in treating disorders of the joints, bones, and muscles. Often, a
rheumatologist diagnoses and treats patients with Raynaud's, but internists and
family practice doctors also are able to diagnose and treat Raynaud's.
Diagnostic Tests and Procedures
To help diagnose Raynaud's, doctors may do a cold
simulation test. This test can trigger an attack that is typical of the
disorder. They also may do tests for inflammatory disorders that damage blood
vessels or nerves. Having these conditions along with Raynaud's symptoms makes
it more likely that a patient has secondary Raynaud's disease.
In the cold simulation test, temperature sensors are
taped to the fingers of the hand. The hand is then briefly exposed to the cold,
usually by dunking it in ice water. If the patient likely has Raynaud's, it
will take more than the normal time for the finger temperature to return to
what it was at the start of the test.
Doctors also may do a test called a nailfold
capillaroscopy (KAP-i-lar-OS-ko-pe). For this test, the doctor puts a drop of
oil on the skin at the base of the fingernail and then looks at it under a
microscope. If the doctor sees abnormal looking blood vessels, this suggests an
inflammatory disorder such as
scleroderma.
Doctors also can use two specific blood tests to
look for inflammatory conditions: the antinuclear antibody test and the
erythrocyte sedimentation rate.
How Is Raynaud's Disease Treated?
There is no cure for primary or secondary Raynaud's,
but many measures can reduce the number or intensity of attacks, including:
- Lifestyle changes
- Medicines
- Treatments for the disease or condition that may
help cause secondary Raynaud's
- Surgery for the tissue damage that some people
with secondary Raynaud's develop
In most people with primary Raynaud's, the disorder
is successfully managed with lifestyle adjustments. Patients with secondary
Raynaud's may need medicines in addition to lifestyle changes, and in rare
cases, they may need surgery. Anyone with Raynaud's who develops sores on their
fingers or toes or elsewhere on their body should see a doctor right away to
prevent tissue loss.
Lifestyle Changes
Most of the lifestyle changes that help people with
Raynaud's aim to avoid the triggers of attacks. These triggers include cold,
emotional stress, and certain medicines, chemicals, or actions. To protect the
body from cold, people can:
- Wear a hat, gloves, scarf, and a coat with snug
cuffs during cold weather.
- Wear gloves or mittens when taking food out of
the refrigerator or freezer.
- Turn down air conditioning, or dress warmly while
in an air conditioned space.
- Warm up the car before driving in cold
weather.
To avoid emotional triggers, people can steer clear
of stressful situations if possible. Relaxation techniques also can be helpful
under stress.
To avoid workplace or recreational triggers, people
can:
- Limit use of vibrating tools.
- Wear proper protective gear if they work with
industrial chemicals.
- Limit frequent and repeated actions of the hands,
such as typing or playing the piano.
A number of medicines can trigger attacks. People
with Raynaud's should avoid:
- Beta blockers
- Over-the-counter cold or allergy remedies or diet
aids that narrow blood vessels
- Birth control pills, which affect blood flow
- Headache medicines that contain ergotamine
Other helpful lifestyle changes for people with
Raynaud's are those that boost blood flow in the body. These include exercising
regularly and quitting smoking.
When attacks do occur, people with Raynaud's can
take several steps to limit the length and strength of the attacks. These steps
include:
- Moving to a warmer spot, such as indoors during
cold weather.
- Warming the hands or feet. Hands can be placed
under the armpits, and feet or hands can be soaked in warm water.
- Wiggling or massaging the fingers and toes.
- Moving the arms in circles or shaking arms or
feet.
- Relaxing and getting out of stressful situations
that trigger the attacks.
Medicines and Surgery
Most of the medicines used to treat people with
Raynaud's are given to improve blood flow to the extremities. These medicines
include calcium channel-blockers, such as:
- Nifedipine
- Amlodipine
- Diltiazem
- Felodipine
- Isradipine
Calcium channel-blockers help limit the number and
severity of attacks in about 2 out of 3 patients with Raynaud's.
Also helpful are alpha-blockers, such as prazosin
and doxazosin. In addition, skin creams that dilate blood vessels, such as
nitroglycerine paste, can help heal skin sores.
The rare patient who develops sores or tissue death
(gangrene) needs more aggressive treatment. Such treatment includes antibiotics
and surgery to cut out damaged tissue. People with severe, worsening Raynaud's
may have surgery or shots to block the action of nerves in the hands and feet
that control blood flow in the skin. This surgery often gets rid of symptoms
for 1–2 years. Patients may need shots more than once.
Living With Raynaud's Disease
Primary and secondary Raynaud's are conditions that
may be lifelong. Most people with primary Raynaud's respond to simple lifestyle
changes or medicines. Such treatment is not always as successful in secondary
Raynaud's. If you have secondary Raynaud's, you may find that in time your
medicines are less effective and your attacks become more frequent and/or more
severe. Switching to a new treatment may help relieve or prevent your symptoms.
Be sure to seek a doctor's care if you develop sores on your fingers or toes or
elsewhere on the body.
Key Points
- Raynaud's is a rare disorder that affects blood
flow to the fingers and toes, and rarely affects other areas such as the nose,
ears, nipples, and lips.
- When Raynaud's occurs without any known cause, it
is called primary Raynaud's. When a cause can be identified, the disease is
called secondary Raynaud's.
- People with Raynaud's have attacks in response to
cold or emotional stress. During these attacks, their fingers, toes, or other
extremities temporarily pale and/or become bluish due to a lack of blood flow.
As blood flow returns, the area turns red. The affected areas also may throb or
feel cold, numb, or tingly.
- Raynaud's is usually diagnosed based on a history
of symptoms, the exclusion of other conditions, and certain diagnostic
tests.
- Common causes of secondary Raynaud's include
other disorders, medicines, chemicals, or work conditions that affect blood
flow to the extremities.
- Primary Raynaud's often can be managed with minor
lifestyle changes. Secondary Raynaud's may require changing or stopping certain
medicines, treating any underlying conditions, and having surgery (in severe
cases).
- People with severe secondary Raynaud's may
develop sores or tissue death (gangrene) in the extremities. This is
uncommon.
Links to Other Information About Raynaud's
Disease
Non-NHLBI Resources
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