Raynaud's Disease

Raynaud’s phenomenon, also called Raynaud's disease, is a condition that affects the blood vessels in the extremities. It is characterized by attacks, called vasospasms, causing 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% of people with Raynaud's.

Fingers of a patient with Raynaud's disease. Source: Wikimedia Commons

This phenomenon usually occurs in response to cold temperatures and/or emotional stress. During these vasospasms, 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).

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. But 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°F

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.

Types

Primary Raynaud's

When the condition occurs on its own and is not associated with any other disease, it is called Raynaud's disease, primary Raynaud’s phenomenon, or idiopathic Raynaud’s phenomenon. There is no known cause for primary Raynaud’s phenomenon. It is more common than the secondary form and often is so mild the patient never seeks medical attention. It generally is an annoyance that causes little disability.

Secondary Raynaud's

When it occurs with another condition such as scleroderma or lupus, it is called Raynaud's phenomenon, or secondary Raynaud’s. Primary Raynaud's is more common and tends to be less severe than secondary Raynaud's. Secondary Raynaud’s phenomenon is a more complex and serious disorder.^[1]

The most common cause of secondary Raynaud’s phenomenon is connective tissue disease. The condition most commonly occurs with scleroderma or lupus, but is also associated with Sjögren’s Syndrome, dermatomyositis, and polymyositis. Some of these diseases reduce blood flow to the fingers and toes by causing blood vessel walls to thicken and the vessels to constrict too easily.

Other possible causes of secondary Raynaud’s phenomenon are carpal tunnel syndrome and obstructive arterial disease (blood vessel disease). Some drugs are also linked to Raynaud’s phenomenon. They include beta-blockers, such as metoprolol or carteolol, used to treat high blood pressure; ergotamine preparations, such as Cafergot or Wigrane, used for migraine headaches; certain agents used in cancer chemotherapy; and drugs, such as over-the-counter cold medication and narcotics, that cause vasoconstriction. Severe cases of Raynaud’s phenomenon—usually of the secondary form—can lead to problems such as skin ulcers (sores) or gangrene (tissue death) in the fingers and toes, which can be painful and difficult to treat.

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, for example, 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.

Causes

The cause of primary Raynaud's disease is unknown.

Secondary Raynaud's can be linked to many different medical or workplace conditions.

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 one out of three people with lupus have Raynaud's. The disease also is linked to other diseases that damage blood vessels or nerves, including:

• Rheumatoid arthritis
• Atherosclerosis
• Sjogren's syndrome
• Dermatomyositis
• Polymyositis
• Buerger's disease

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 a person 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 people 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.

Chances of Developing Raynaud's Disease

About three out of four 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.

Although estimates vary, most studies show that Raynaud’s phenomenon affects about 3% of the general population. For most, the symptoms are mild and not associated with any blood vessel or tissue damage.

Most people with Raynaud’s phenomenon have the primary form, which is not associated with any underlying disease. In fact, in these individuals it is thought to be an exaggeration of normal responses to cold temperature and/or stress.

When Raynaud’s phenomenon is caused by or associated with an underlying disease, it is referred to as secondary Raynaud’s phenomenon. Secondary Raynaud’s phenomenon tends to begin later in life than the primary form, typically after 35 to 40 years of age.

It is common for patients with a connective tissue disease to have Raynaud’s phenomenon. It occurs in more than 90% of patients with scleroderma, and in about 30% of patients with systemic lupus erythematosus and with Sjögren’s syndrome. Secondary Raynaud’s phenomenon may also be associated with exposure to vibrating tools such as jackhammers, which cause trauma to the hands and wrists. And it may be linked to certain drugs, such as chemotherapy agents, or to chemicals such as vinyl chloride.

Signs and Symptoms

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.

Once an attack begins, a person may experience three phases (though not all people have all three) of skin color changes—typically from white to blue to red—in the fingers or toes. Whiteness (called pallor) may occur in response to spasms of the arterioles (small branches of an artery) and the resulting collapse of the arteries supplying the fingers and toes. Blueness (cyanosis) may appear because the fingers or toes are not getting enough oxygen-rich blood. Finally, as the arterioles dilate (relax) and blood returns to the digits, redness (rubor) may occur.

During the attack, the fingers or toes may feel cold and numb as blood flow to them is interrupted. As the attack ends and blood flow returns, fingers or toes may throb and tingle. Typically, the blood flow to the skin will remain low until the skin is rewarmed. After warming, it usually takes 15 minutes to recover normal blood flow to the skin.

Diagnosis

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 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

Physicians can now distinguish primary from secondary Raynaud’s phenomenon with a complete history and physical examination. Sometimes, special blood testing is needed. If the evaluation and special testing studies are normal, then the diagnosis of primary Raynaud’s phenomenon can be made and it is unlikely to change into a secondary form. Interestingly, about 30% of first-degree relatives of patients with primary Raynaud’s phenomenon also have the condition. This finding suggests that primary Raynaud’s phenomenon is determined by some yet-to-be discovered genetic trait.

A few tests can help the doctor distinguish between primary and secondary Raynaud’s phenomenon. They include the following:

Nailfold capillaroscopy. 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 problems in the tiny blood vessels called capillaries. If the capillaries are enlarged or malformed, the patient may have a connective tissue disease.^[2]

Antinuclear antibody (ANA) test. In this blood test, the doctor determines whether the body is producing special proteins called antibodies that are directed against the nuclei of the body’s cells. These abnormal antibodies are often found in people who have connective tissue diseases or other autoimmune disorders.

Erythrocyte sedimentation rate (ESR or sed rate). This is a laboratory test for inflammation that measures how quickly red blood cells fall to the bottom of a test tube of unclotted blood. Rapidly descending cells (an elevated sed rate) indicate inflammation in the body.

Treatment

There is no cure for primary or secondary Raynaud's, but the following measures can reduce the number or intensity of attacks.

• 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.

Holistic and alternative treatments

The following nondrug treatments and self-help measures can decrease the severity of Raynaud’s attacks and promote overall well-being.

• Take action during an attack. Both the length and severity of an attack can be decreased by a few simple actions. The first and most important one is to avoid the cold. Warming the body and the hands or feet is also helpful. Run warm water over the fingers or toes or soak them in a bowl of warm water to warm them. If a stressful situation triggers the attack, get out of the stressful situation, if possible, and relax. While biofeedback and similar nondrug methods are used, formal studies have suggested they are not helpful.
• Keep warm. It is important not only to keep the extremities warm but also to avoid chilling any part of the body. Remember, a drop in the body’s core temperature triggers the attack. Shifting temperature (for example, rapidly moving from 90°F outside to a 70°F air-conditioned room) and damp rainy weather are to be avoided. In cold weather, 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. Keep feet warm and dry. Some people find it helpful to wear mittens and socks to bed during the 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.

In warm weather, air conditioning also can trigger attacks. Setting the thermostat for a higher temperature or wearing a sweater indoors can help prevent an attack. Some people find it helpful to use insulated drinking glasses and to put on gloves before handling frozen or refrigerated foods.

• Avoiding smoking. The nicotine in cigarettes causes the skin temperature to drop, which may lead to an attack.
• Avoiding aggravating medications such as vasconstrictors, which cause the blood vessels to narrow. Vasoconstrictors include beta-blockers, many cold preparations, caffeine, narcotics, some migraine headache medications, some chemotherapeutic drugs, and clonidine, a blood pressure medication. Some studies also associate the use of estrogen with Raynaud’s phenomenon.
• Controlling 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 can help decrease the number and severity of attacks. Local hospitals and other community organizations, such as schools, often offer programs in stress management.
• Exercising regularly. 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 cardiovascular fitness and restful sleep. Patients with Raynaud’s phenomenon should talk to their doctors before starting an exercise program.
• Seeing 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 episodes occur only on one side of the body (one hand or one foot) and any time one results in sores or ulcers on the fingers or toes.

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 two out of three 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 one to two years. Patients may need shots more than once.

Living With Raynaud's Disease

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. 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. People with secondary Raynaud's may find that in time their medicines become less effective and their attacks become more frequent and/or more severe. Switching to a new treatment sometimes helps to relieve or prevent symptoms. Patients need also to be vigilant in watching for sores on the fingers or toes or elsewhere on the body which may become gangrenous and therefore need urgent medical evaluation.

Research

Researchers are studying techniques such as laser Doppler imaging to better diagnose Raynaud’s phenomenon and to predict and monitor its course and responsiveness to treatment.

They are also evaluating the use of new treatments to improve blood flow for those who have Raynaud’s phenomenon. These include the high blood pressure drug losartan (Cozaar); prostaglandins such as iloprost and alprostadil (Caverject, Edex); the male erectile dysfunction drug sildenafil (Viagra); the blood-clot-preventing drug ticlopidine (Ticlid); and the herbal remedy gingko biloba.

Treatments such as L-arginine, taken orally, have been studied as a way to reverse Raynaud’s-related damage to tissue in the toes and fingers, but they have been found ineffective in most studies.

Basic investigators are studying the molecular mechanisms behind Raynaud’s phenomenon, the anatomy of blood vessels, and possible genetic associations. Researchers in scleroderma and other connective tissue diseases are also investigating Raynaud’s phenomenon in relation to these diseases.

References

1. ↑ Raynaud's Association
2. ↑ Anders H, Sigl T, Schattenkirchner M. Differentiation between primary and secondary Raynaud's phenomenon: a prospective study comparing nailfold capillaroscopy using an ophthalmoscope or stereomicroscope.] Ann Rheum Dis. 2001 April;60(4):407–409. PDF

External Links

Arthritis Foundation: Raynaud's Phenomenon

Vascular Disease Foundation: Raynaud's Disease: What Is It?

Cedars-Sinai Health System: Raynaud's Phenomenon