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Raynaud's Disease, Phenomenon & Syndrome: Symptoms, Causes, Treatment

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Raynaud’s phenomenon (also called Raynaud’s disease or Raynaud’s syndrome) is a disorder that affects the blood vessels in the fingers and toes. Blood vessels in the nose, lips or ear lobes may also be affected.

This disorder is characterized by episodic spasms, called vasospastic attacks, which cause the small blood vessels in the fingers and toes to constrict (tighten or close) in response to temperature extremes, certain occupational exposures, or excitement. With Raynaud’s, the skin on the affected areas becomes white or bluish and cold or numb.

Raynaud’s phenomenon

Raynaud’s phenomenon can occur on its own (primary Raynaud’s phenomenon), or it can be related to another medical condition (secondary Raynaud’s phenomenon).

Primary Raynaud’s Phenomenon

Also known as Raynaud’s disease, primary Raynaud’s phenomenon is the most common and the milder of the two types. A person who has primary Raynaud’s has no other diseases or associated medical problems that may cause Raynaud’s symptoms. About 75 percent of all cases of primary Raynaud’s phenomenon are diagnosed in women between ages 15 and 40. People with the primary form rarely develop the other diseases associated with Raynaud’s such as lupus or scleroderma.

Secondary Raynaud’s Phenomenon

Less common than the primary form, however, secondary Raynaud’s phenomenon is often a more serious disorder. Secondary Raynaud’s is caused by an underlying disease or condition. It is especially common in people with connective tissue diseases. Some of these diseases reduce blood flow to the fingers and toes by causing the blood vessel walls to thicken and the vessels to constrict too easily. Raynaud’s phenomenon occurs in about 85 to 95 percent of patients with scleroderma and is present in about one-third of patients with systemic lupus erythematosus (lupus). Raynaud’s also can occur in patients who have other connective tissue diseases, including Sjögren’s syndrome, dermatomyositis and polymyositis.

Other possible causes of secondary Raynaud’s phenomenon include:

  • Traumatic vasospasm from vibrating tools or repeated pounding with the palm of the hand (hyothenar hammer syndrome)
  • Carpal tunnel syndrome
  • Obstructive arterial disease (blood vessel disease)
  • Some medications, including beta-blockers, ergotamine preparations, certain chemotherapy drugs, and those that cause vasoconstriction (such as some over-the-counter cold medications and narcotics)
  • Thyroid disorders

Who is affected by Raynaud’s?

Although estimates vary, recent surveys show that Raynaud’s phenomenon may affect 3 to 5 percent of the general population in the United States. Women are more likely than men to have the disorder. Raynaud’s phenomenon also appears to be more common in people who live in colder climates. However, people with the disorder who live in mild climates may have more attacks during colder weather. About 25 percent of people with Raynaud’s have a family history of the condition.

What causes Raynaud’s?

An attack of Raynaud’s is usually triggered by exposure to cold or emotional stress.

Under normal circumstances, when a person is exposed to cold, his or her body’s response is to slow the loss of heat. The body does this by causing the blood vessels that control blood flow to the skin’s surface to move blood from the surface arteries to veins deeper in the body.

For people who have Raynaud’s, however, this normal body response is intensified by contractions of the small blood vessels that supply blood to the fingers and toes.

In some cases, this causes the arteries of the fingers and toes to collapse or constrict. The result is a greatly decreased supply of blood to the affected body areas, causing skin discoloration.

Symptoms of Raynaud's

A person with Raynaud's phenomenon can experience three phases of skin color changes: white (pallor), blue (cyanosis) and red (rubor). There is not a set order to the changes in skin color and not all people experience all three skin colorations.

  • Pallor (whiteness) may occur in response to the collapse of the arteries in an affected body part.
  • Cyanosis (blueness) appears because the fingers or toes are not getting enough oxygen-rich blood. Other symptoms that occur during cyanosis are feeling cold and numbness.
  • Rubor (redness) occurs as the blood returns to the affected areas. After an attack is over, throbbing and tingling may occur in the fingers and toes. Attacks of Raynaud's Phenomenon can last from less than a minute to several hours.

How is Raynaud’s diagnosed?

If a doctor suspects Raynaud’s phenomenon, he or she will thoroughly evaluate the patient’s medical history and perform a complete physical exam to rule out other medical problems. Often, it is fairly easy to diagnose Raynaud’s, but more difficult to identify the form of the disorder.

One diagnostic test useful in helping doctors determine the correct form of Raynaud’s is known as a nailfold capillaroscopy, in which capillaries are studied under a microscope. For people with Primary Raynaud’s Phenomenon, the results of this test will be normal. The results of this test will be abnormal for those who have the secondary form.

During a nailfold capillaroscopy, the doctor places a drop of oil on the patient’s nailfold - the skin at the base of the fingernail. The doctor will then examine the nailfold under a microscope or a hand-held ophthalmoscope to look for abnormalities of the capillaries. If the capillaries are enlarged or abnormal, this may indicate that the patient has a connective tissue disease.

If the patient has symptoms suggesting secondary Raynaud’s phenomenon, the doctor may order a complete blood count (CBC), a urinalysis, and chemistry profile.

Other tests a doctor may order to help distinguish between the two forms of Raynaud’s are the antinuclear antibody test (ANA), the erythrocyte sedimentation rate (ESR) or tests for the rheumatoid factor and complement levels.

How is Raynaud’s treated?

The goals of treatment are to reduce the severity of attacks and to prevent tissue damage and loss in the fingers and toes. Doctors may prescribe medications for some patients - usually those with Secondary Raynaud’s phenomenon; however, doctors most often prescribe non-drug treatments.

Several non-drug treatments, described below, may help decrease the severity of a Raynaud’s attack as well as promote overall well-being.

  • Be proactive during an attack: A Raynaud’s attack should not be ignored. By taking the proper steps, the length and severity of the attack can be decreased. The first and most important action is to warm your hands and feet. In cold weather, you should go indoors. Running warm water over your fingers and toes or soaking them in a bowl of warm water will also help. Learning relaxation techniques as well as taking time to relax will further help to end an attack.
  • Keep warm: Not only is it important to keep your hands and feet warm, but it is also helpful to avoid chilling any other part of the body. In cold weather, pay particular attention to the way you dress. Several layers of loose clothing, socks, hats, and gloves or mittens are recommended. Hats are particularly important because a great deal of body heat is lost through the scalp.
    • Keep your feet and hands dry and warm. 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. Other suggestions include:
      • Avoid touching cold metals
      • Avoid putting hands in cold water or holding iced drinks
      • Avoid air-conditioned rooms or the frozen food sections of grocery stores as much as possible
  • Practice good skin care: Prevent dryness or cracking of the skin by applying a liberal amount of moisturizer or hand cream, especially after washing your hands.
  • Quit smoking: Nicotine causes the skin temperature to drop, which may lead to an attack.
  • Learn to manage stress: Because stress 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 exercises or biofeedback training can help decrease the number and the severity of attacks.
  • Exercise: Many doctors encourage patients who have Raynaud’s phenomenon to exercise regularly. Most people find that exercise promotes an overall well-being, increases energy level, helps control weight, and promotes restful sleep. Patients with Secondary Raynaud’s should talk to a doctor before exercising outdoors in cold weather. Always talk to your doctor before starting an exercise program.
  • See a doctor: You should see your doctor if you are worried or frightened about attacks or if you have questions about caring for yourself. Always see your doctor 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.
  • Medications: People with secondary Raynaud's phenomenon are more likely than those with the primary form to be treated with medications. Many health care professionals believe that the most effective and safest drugs are calcium- channel blockers, which relax smooth muscles and dilate the small blood vessels. These drugs decrease the frequency and severity of attacks in about two-thirds of patients who have Primary or Secondary Raynaud's phenomenon. These drugs also can help heal skin ulcers on the fingers or toes.

Other medications that have helped patients with Raynaud’s include alpha-blockers, which counteract norepinephrine, a hormone that constricts blood vessels, and vasodilators (drugs that relax the blood vessels), such as nitroglycerine paste, which is applied to the fingers, to help heal skin ulcers.

It is important to note that treatment with medications is not always successful. Often, patients with the secondary form of Raynaud’s 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 and some people may experience side effects that require stopping the medication. For other people, a drug may become less effective over time.

Regardless of the medication a patient is using, it is important that he or she schedule follow-up appointments with his or her doctor so the effects of the medications can be monitored.

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