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What is Raynaudís Phenomenon?
Raynaudís Phenomenon is a group of related symptoms that are the result of the constriction of small blood vessels called arterioles. This constriction, known as vasospasm or a vasospastic attack, can occur when someone is exposed to cold or during emotional distress, and can last anywhere from a few minutes to several hours. The fingers are most commonly affected and to a lesser extent the toes. However, the earlobes, nose, lips, and tongue can all be affected as well.
What are the Symptoms?
When these vasospastic attacks occur there are three subsets of symptoms: Change in skin color, altered sensation, and tissue damage.
Change in Skin Color Ė This is most noticeable in the fingers as they are more easily observed but it can occur in any of the areas affected by Raynaudís Phenomenon. There is normally a three stage cycle that occurs during an attack: white Ė blue - red. When a person experiences Raynaudís Phenomenon due to exposure to cold or as the result of emotional episode the hands will quickly turn pale (pallor). This is a result of decreased blood flow to the area. This decreased blood flow results in less oxygen getting to the tissue causing the area to turn blue (cyanosis). When the blood vessels relax (as the body warms up or as emotions settle down) the body will compensate for the previous lack of blood flow by increasing the blood flow to more than normal (compensatory hyperemia). This will cause the area to look red and blushed (rubor).
Altered Sensation Ė Often the affected area will feel cold shortly after the episode begins. Then as it progresses the area will sometimes become painful and in extreme cases may even become numb. As blood-flow returns to the area tingling or a painful throbbing sensation may be felt.
Tissue Damage Ė Tissue damage only occurs in severe cases of Raynaudís Phenomenon. Prolonged periods without oxygen can cause cells to die (necrosis). The tissue damage can present in several ways and is almost always seen on the hands and toes. Often times the fingers will become thin and the skin in the hands will become smooth and shiny. Also, sores can develop on the fingers and toes. These can become infected easily and are usually painful.
What Causes Raynaudís Phenomenon and Who is at Risk?
Raynaudís Phenomenon is a fairly common disorder affecting about 5-10% of the population. Raynaudís Phenomenon can have many different underlying causes. Many different connective tissue diseases including Lupus, Rheumatoid Arthritis, Dermatomyositis, Sjogrenís Syndrome, Polymyositis, and Scleroderma can cause Raynaudís Phenomenon. It is estimated that one third of people with Lupus and Sjogrenís Syndrome, and 90% of people with Scleroderma will develop Raynaudís Phenomenon. Other diseases can cause Raynaudís Phenomenon including Carpal Tunnel Syndrome and Obstructive Arterial Disease. Hormonal imbalances like Hypothyroidism and Carcinoid can lead to Raynaudís Phenomenon as well. People who use vibrating machinery, like jackhammers, on a regular basis can also develop Raynaudís Phenomenon. Finally, frostbite can leave long term blood vessel damage that may lead to Raynaudís Phenomenon.
Raynaudís Phenomenon can also have chemical causes. Smoking increases your risk of experiencing Raynaudís Phenomenon because nicotine constricts blood vessels, predisposing you to the condition. People on certain blood-pressure medications called beta blockers, or ergotamine preparations, used to treat migraines, are more likely to experience Raynaudís Phenomenon. The use of estrogens without supplemental progesterone has been linked to Raynaudís Phenomenon as well. Over the counter cold medicines, narcotics, and certain cancer drugs can also induce Raynaudís Phenomenon. Workers in some plastic factories who are exposed to vinyl chloride can develop a Scleroderma-like illness which can cause Raynaudís Phenomenon.
These cases of Raynaudís Phenomenon which have an underlying cause are called Secondary Raynaudís Phenomenon. These cases are usually more severe, especially those cases which occur as a result of a connective tissue disease.
There are cases however, mostly seen in women ages 15-40 in which there is no underlying cause. This is called Primary Raynaudís Phenomenon and the symptoms are usually much less severe. Tissue damage in Primary Raynaudís Phenomenon is much rarer than it is in Secondary Raynaudís Phenomenon.
How is Raynaudís Phenomenon Diagnosed?
It is important to remember that only your doctor can diagnose Raynaudís Phenomenon. If you experience any of the symptoms of Raynaudís Phenomenon you should consult your doctor so that he or she may diagnose the problem.
While a simple history may be enough to diagnose Raynaudís Phenomenon, more detailed tests may be necessary to determine which category (Primary or Secondary) it falls into.
Once your doctor has diagnosed you as having experienced Raynaudís Phenomenon, has ruled out any chemical or work related causes, and has examined your hands for sores, ulcers, or other tissue damage, he or she may do a series of tests to determine whether you have Primary or Secondary Raynaudís Phenomenon.
One of the first tests your doctor may do is known as Nailfold Capillaroscopy. This test is a study of the capillaries at the base of the fingernails to determine whether or not they are of normal size and shape. To do this study the doctor will place a drop of oil on the nailfold (the point where the skin and fingernail meet). Then the doctor will examine the nailfold under a microscope in order to view the capillaries (the smallest of blood vessels). Enlarged or deformed capillaries are a sign of connective tissue disease.
Blood tests may also be required in order to determine whether you have Primary or Secondary Raynaudís Phenomenon. An antinuclear antibody test (ANA test) can be done in order to detect antibodies (immune system proteins). The presence of these antibodies is a sign of autoimmune diseases like Lupus which could be causing Raynaudís Phenomenon. Another blood test that the doctor may do is an erythrocyte sedimentation rate (sed rate). A sed rate will determine how fast the red blood cells in your body will settle to the bottom of vial containing unclotted blood. This test will measure the amount of inflammation in the body which can be a sign of various diseases that may cause Raynaudís Phenomenon. A T4 and TSH test, which measures how well the thyroid is functioning, may be useful in ruling out hypothyroidism as a cause of Raynaudís Phenomenon. Similarly a Rheumatoid Factor test can be done to determine if certain connective tissue diseases are causing the symptoms.
If the doctor performs these tests and discovers no sores or ulcers, normal capillaries, and normal results from the blood tests, then you likely have Primary Raynaudís Phenomenon. However, a positive result on any of these tests may result in a diagnosis of Secondary Raynaudís Phenomenon. In this case your doctor may wish to do further tests in order to better manage the underlying ailment in order to alleviate symptoms or at the very least prevent them from worsening.
Once Raynaudís Phenomenon is diagnosed it can be further classified by the severity of the symptoms. The Taylor-Pelmear Scale was originally designed to categorize Raynaudís Phenomenon in workers who developed it from using vibrating tools. However, it is a useful tool for determining if the condition is improving or worsening and verifying the effectiveness of treatment. The Taylor-Pemear Scale classifies Raynaudís Phenomenon into four categories.
How is Raynaudís Phenomenon Treated?
There are two methods for treating Raynaudís Phenomenon: lifestyle change and medication. Only your doctor can prescribe drugs, but lifestyle changes, the main method for treating Raynaudís Phenomenon, are simple things that you can do on your own. Below are some simple measures that you can take on your own to help make attacks less severe and less frequent.
Keep Warm: Wearing mittens and heavy socks can keep your fingers and toes warm in cold weather preventing episodes of vasospasm. In some people, reaching into the freezer can cause an episode. In cases like this oven mitts or a towel can be used to keep the fingers warmer. Also, once an attack has begun, quickly warming up the hands can shorten the length and reduce the severity of the attack.
Stop Smoking: Nicotine can make your blood vessels more narrow worsening the symptoms of Raynaudís Phenomenon. Smoking can also lower your skin temperature increasing the likelihood of an attack.
Control Stress: The severe emotional fluctuations that result from stress can cause Raynaudís Phenomenon and can increase the frequency of attacks. Taking time out to relax, deep breathing, and other techniques can be employed to help control stress. Support groups also exist to help people who suffer from stress and anxiety. Taking a moment to relax during an attack can make the attack shorter and less severe.
Exercise: Exercise can help to reduce stress and also promotes blood flow which can help prevent Raynaudís Phenomenon.
In more severe cases of Raynaudís Phenomenon your doctor may suggest medication to help the condition. One class of drugs that seems to be most effective in helping people who experience Raynaudís Phenomenon are called calcium channel blockers. These drugs help to dilate and relax the blood vessels and prevent them from constricting. This can help prevent symptoms and help to heal the sores and ulcers seen in Secondary Raynaudís Phenomenon. Another class of drugs that may help are called alpha-blockers. These drugs prevent the hormone norepinepherine from constricting blood vessels. Doctors may also prescribe a topical paste that contains nitroglycerine to relax the blood vessels in your hand for use during an attack. Low doses of blood thinners like aspirin can also be helpful in treating Raynaud's Phenomenon.
Some research also supports the use of natural therapies either in conjunction with the lifestyle changes and medications above. Vitamin E may help improve circulation. Calcium and Magnesium supplements may be beneficial in the treatment of Raynaudís Phenomenon by preventing spasms.
In the most severe of cases where the associated tissue damage may result in the loss of a finger or toe doctors may consider a surgical procedure called a sympathectomy. In this procedure the nerves that are responsible for causing the blood vessels to constrict, are removed through small incisions at the base of the fingers.
Hope on the Horizon
There is considerable research being done on Raynaudís Phenomenon. Some Prostaglandins like iloprost and alprostadil are being studied for the potential to lower blood pressure by relaxing smooth muscle. This could dilate the blood vessels and prevent vasospasm. Another blood vessel drug called lostartan may be helpful as well. Finally, nitric oxide is known to dilate blood vessels. New topical drugs that can release nitric oxide locally (only to the area in which the ointment is applied) are being studied for the potential to treat Raynaudís Phenomenon. This along with research on many of the connective tissue diseases that can cause Raynaudís Phenomenon offer a great deal of hope to those who suffer from this affliction.
Authors: Peter Mazari, BA, Robert Wood Johnson Medical School, Piscataway, NJ
Zinovy Meyler, D.O., New York-Presbyterian Hospital, The University Hospital of Columbia and Cornell