Raynaud's Syndrome

horizontal rule

1. What is Raynaud's Syndrome?
Raynaud's syndrome is a disorder that affects the small blood vessels in the fingers, toes, ears, and nose. It is characterized by episodes when the blood vessels in the fingers and toes become narrow. Raynaud's syndrome affects up to 5 to 10 percent of the population to some degree. Women are more likely than men to have the disorder and it is more common in colder weather.

2. What happens during an attack?
An attack is usually triggered by exposure to cold or emotional stress and can last less than a minute or up to several hours.  When a person is exposed to cold, the normal response is to reduce heat loss by reducing the blood flow to the skin surface.  In Raynaud's syndrome, this normal response to cold is intensified and small blood vessels in the coldest parts of the skin (e.g. fingers and toes) go into spasm.  As a result, the blood supply to these parts of the skin is greatly decreased and this is evident by the skin colour change and other symptoms.

Once the attack begins, there are usually three phases of skin colour change: white, blue, and then red.  The order of the change in skin colour is not the same for all people, and not everyone has all three.

bulletPallor (whiteness) occurs in response to the narrowing of the small arteries by spasm.
bulletCyanosis (blueness) may appear because the skin is not getting enough oxygen-rich blood. The skin may also feel cold and numb.
bulletRubor (redness) as the blood vessels eventually relax the blood returns to the digits. Throbbing and tingling may occur in the fingers and toes.

3. How Is Raynaud's Syndrome classified?
Raynaud's is classified into two forms depending on if there is any associated conditions which are believed to trigger the Raynaud's

bulletPrimary Raynaud's
Most people have the milder primary form of Raynaud's syndrome for which there is no obvious cause. More women than men are affected, and approximately 75 percent of all cases are diagnosed between the ages of 15 and 40 years.  Some people who have primary Raynaud's for several years later develop a connective tissue disorder such as scleroderma. Less than one in ten develop a secondary disease.
bulletSecondary Raynaud's
Much less common than the primary form but 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.  Secondary Raynaud's 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 can occur with other connective tissue diseases, including Sjögren's syndrome, dermatomyositis, and polymyositis.

Other possible causes of secondary Raynaud's are carpal tunnel syndrome and arterial disease.  Drugs, including beta-blockers, ergotamine preparations, agents used in cancer chemotherapy, and drugs that cause vasoconstriction (e.g. 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. Workers in the plastics industry who are exposed to vinyl chloride can 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. In addition, people whose fingers are subject to repeated stress, such as typing or playing the piano, are more vulnerable to the disorder.

People with secondary Raynaud's often experience associated medical problems. The more serious problems are skin ulcers or gangrene of 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 gullet and can occur in people with connective tissue diseases, particularly scleroderma.

4. How do you diagnose Raynaud's Syndrome
The description of typical attacks is usually enough to suggest the diagnosis. It is more difficult to decide if it is the primary or secondary form and this requires a physical examination and some blood tests.  There are some tests that are only available to specialists:

bulletNailfold capillaroscopy can help to distinguish between primary and secondary Raynaud's. During this test, the doctor puts a drop of oil on the skin at the base of the fingernail and then examines the blood vessels in the skin under a microscope. If the capillaries are enlarged or deformed, the patient may have a connective tissue disease.
bulletThe doctor may also order some blood tests, an antinuclear antibody (ANA) test and an erythrocyte sedimentation rate (ESR). The ANA test is often positive in people who have connective tissue diseases or other autoimmune disorders. The ESR test is a measure of inflammation in the body.

5. Diagnostic Criteria for Raynaud's Syndrome

Primary Raynaud's

bulletPeriodic vasospastic attacks of pallor or cyanosis for at least 2 years
bulletNormal nailfold capillary pattern
bulletNegative antinuclear antibody test
bulletNormal erythrocyte sedimentation rate
bulletAbsence of pitting scars or ulcers of the skin, or gangrene in the fingers or toes

Secondary Raynaud's

bulletPeriodic vasospastic attacks of pallor and cyanosis
bulletAbnormal nailfold capillary pattern
bulletPositive antinuclear antibody test
bulletAbnormal erythrocyte sedimentation rate
bulletPresence of pitting scars or ulcers of the skin, or gangrene in the fingers or toes

6. What is the treatment for Raynaud's Syndrome?
There is no specific treatment that cures Raynaud's.  The main aim therefore is to reduce the number and severity of attacks and to prevent tissue damage and loss in the fingers and toes.  Cconservative (non-drug) treatment and self-help measures are used first.  Drugs may be used for some patients, usually those with secondary Raynaud's in addition to treatment of any underlying disease or condition.

7. What are non-drug treatments and self-help measures?
Non-drug treatments and self-help measures can decrease the severity of Raynaud's attacks and promote overall well-being.

bulletTake action during an attack
The duration and severity of an attack 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 help. 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. 
bulletKeep Warm
It is important to keep the hands and feet warm and to avoid chilling any part of the body. In cold weather, people with Raynaud's 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 head. 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 should talk to their doctors before exercising outdoors in cold weather. People with Raynaud's 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.
bulletStop smoking
The nicotine in cigarettes causes the skin temperature to drop, which may lead to an attack.
bulletControl Stress
Stress and emotional upsets may trigger an attack, particularly for people who have primary Raynaud's. 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.
bulletExercise
Most people find that exercise promotes overall well-being, increases energy level, helps control weight, and promotes restful sleep.
bulletWhen to see a doctor
People with Raynaud's 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.

8. Drug treatment
People with secondary Raynaud's are more likely than those with the primary form to be treated with medications. The most effective and safest drugs are calcium-channel blockers, which relax the smooth muscle in the walls of the blood vessels allowing them to dilate. These drugs decrease the frequency and severity of attacks in about two-thirds of patients who have primary and secondary Raynaud's. These drugs also can help heal skin ulcers on the fingers or toes.

Other patients find relief with drugs called alpha-blockers that counteract the actions of adrenaline, a hormone that constricts blood vessels. Some doctors prescribe a non-specific vasodilator, such as GTN which is applied to the fingers, to help heal skin ulcers. Patients should keep in mind that the treatment for Raynaud's 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 often 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 may affect the growing foetus so women who are pregnant or are trying to become pregnant should avoid taking these medications.

Information