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.

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.

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.

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. 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.

Causes

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:

• Rheumatoid arthritis
• Atherosclerosis
• Sjogren's syndrome, dermatomyositis, and 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 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.

Reference:

National Heart, Lung, and Blood Institute, USA.