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Raynaud's Disease

Definition :
Raynaud's disease is a condition that causes some areas of your body — such as your fingers, toes, tip of your nose and your ears — to feel numb and cool in response to cold temperatures or stress. It's a disorder of the blood vessels that supply blood to your skin. During a Raynaud's attack, these arteries narrow, limiting blood circulation to affected areas.

You can have Raynaud's without any underlying disease associated with it, in which case it's called Raynaud's disease or primary Raynaud's. Or it can be part of another disease, in which case doctors may refer to it as Raynaud's phenomenon or secondary Raynaud's.

Raynaud's disease affects a small percentage of Americans. Women are more likely than men are to have the disorder. It's more common in people who live in colder climates.

Treatment of Raynaud's disease depends on its severity and the presence or absence of associated conditions. For most people, Raynaud's disease is more a nuisance than a disability.

Causes:
Doctors don't completely understand the cause of Raynaud's attacks, but blood vessels in the hands and feet appear to overreact to cold temperatures or stress.

When your body is exposed to cold temperatures, your extremities lose heat. Your body slows down blood supply to your fingers and toes to preserve your body's core temperature. Your body specifically reduces blood flow by narrowing the small arteries under the skin of your extremities. In people with Raynaud's, this normal response is exaggerated. Stress causes a similar reaction to cold in the body, and likewise the body's response may be exaggerated.

With Raynaud's, arteries to your fingers and toes go into what's called vasospasm. This constricts the vessels, dramatically but temporarily limiting blood supply. Over time, these same small arteries may also thicken slightly, further limiting blood flow. The result is that affected skin turns a pale and dusky color due to the lack of blood flow to the area. Once the spasms subside and blood returns to the area, the tissue may turn red before returning to a normal color.

Cold temperatures are most likely to provoke an attack. Exposure to cold can be as simple as putting your hands under a faucet of running cold water, taking something out of the freezer or exposure to cold air. For some people, exposure to cold temperatures isn't necessary. Emotional stress alone can cause an episode of Raynaud's.

Some researchers are studying whether Raynaud's may be partly an inherited disorder.

Primary vs. secondary Raynaud's
Raynaud's occurs in two main types :

  • Primary Raynaud's. This is Raynaud's without an underlying disease or associated medical problem that could provoke vasospasm. Also called Raynaud's disease, it's the most common form of the disorder. Primary Raynaud's typically affects the digits of both hands and both feet.
  • Secondary Raynaud's. This is Raynaud's caused by an underlying problem. Also called Raynaud's phenomenon, secondary Raynaud's usually affects both of your hands or both feet. Although secondary Raynaud's is less common than the primary form, it's often a more complex and serious disorder.

Causes of secondary Raynaud's include :

  • Scleroderma. Raynaud's phenomenon occurs in the majority of people who have scleroderma — a rare disease that leads to hardening and scarring of the skin. Scleroderma, a type of connective tissue disease, results in Raynaud's because the disease reduces blood flow to the extremities. It causes tiny blood vessels in the hands and feet to thicken and to constrict too easily, promoting Raynaud's.
  • Lupus. Raynaud's is also a common problem for people with lupus — an autoimmune disease that can affect many parts of your body, including your skin, joints, organs and blood vessels. An autoimmune disease is one in which your immune system attacks healthy tissue.
  • Rheumatoid arthritis. Raynaud's may be an initial sign of rheumatoid arthritis — an inflammatory condition causing pain and stiffness in the joints, often including the hands and feet.
  • Sjogren's syndrome. Raynaud's phenomenon can also occur in people who have Sjogren's syndrome — a rare disorder that often accompanies scleroderma, lupus or rheumatoid arthritis. The hallmark of Sjogren's syndrome, a connective tissue disease, is chronic dryness of the eyes and mouth.
  • Diseases of the arteries. Raynaud's phenomenon can be associated with various diseases that affect arteries, such as atherosclerosis, which is the gradual buildup of plaques in blood vessels that feed the heart (coronary arteries), or Buerger's disease, a disorder in which the blood vessels of the hands and feet become inflamed. Primary pulmonary hypertension, a type of high blood pressure that affects only the arteries of the lungs, is frequently associated with Raynaud's.
  • Carpal tunnel syndrome. The carpal tunnel is a narrow passageway in your wrist that protects a major nerve to your hand. Carpal tunnel syndrome is a condition in which pressure is put on this nerve, producing numbness and pain in the affected hand. The affected hand may become more susceptible to cold temperatures and episodes of Raynaud's.
  • Repetitive trauma. Raynaud's can also be caused by repetitive trauma that damages nerves serving blood vessels in the hands and feet. In fact, nerve damage is thought to play a role in many cases of Raynaud's. Some people who type or play the piano vigorously or for long periods of time may be susceptible to Raynaud's. Workers who operate vibrating tools can develop a type of Raynaud's phenomenon called vibration-induced white finger.
  • Smoking. Smoking constricts blood vessels and is a potential cause of Raynaud's.
  • Injuries. Prior injuries to the hands or feet, such as wrist fracture, surgery or frostbite, can lead to Raynaud's phenomenon.
  • Certain medications. Some drugs — including beta blockers, which are used to treat high blood pressure; migraine headache medications that contain ergotamine; medications containing estrogen; certain chemotherapy agents; and drugs that cause blood vessels to narrow, such as some over-the-counter cold medications — have been linked to Raynaud's.
  • Chemical exposure. Some workers in the plastics industry who are exposed to vinyl chloride develop an illness similar to scleroderma. Raynaud's can be a part of that illness.
  • Other causes. Raynaud's has also been linked to an underactive thyroid gland (hypothyroidism) and, rarely, to certain cancers.
Risk Factors:
Primary Raynaud's mainly affects women. Although anyone can develop the condition, women between the ages of 15 and 40 are most often affected. The disorder is also more common in people who live in colder climates.

Risk factors for secondary Raynaud's include associated diseases, such as scleroderma and lupus, and smoking.

People in certain occupations, such as workers who operate tools that vibrate, also may be more vulnerable to secondary Raynaud's.

Symptoms :
Raynaud's disease is more than simply having cold hands and cold feet, and it's not the same as frostbite. Signs and symptoms of Raynaud's depend on the frequency, duration and severity of the blood vessel spasms that underlie the disorder. Signs and symptoms include :

  • Sequence of color changes in your skin in response to cold or stress
  • Numb, prickly feeling or stinging pain upon warming or relief of stress.
At first during an attack of Raynaud's, affected areas of your skin usually turn white. Then, the areas often turn blue and feel cold and numb, and your sensory perception is dull. The affected skin may look slightly swollen. As circulation improves, the affected areas may turn red, throb, tingle or swell. The order of the changes of color isn't the same for all people, and not everyone experiences all three

colors.

Occasionally, an attack affects just one or two fingers or toes. Attacks don't necessarily always affect the same digits. Although Raynaud's most commonly affects your fingers and toes, the condition can also affect other areas of your body such as your nose, cheeks, ears and even tongue. An attack may last less than a minute to several hours. Over time, attacks may grow more severe.

People who have Raynaud's accompanied by another disease may also have symptoms related to their underlying condition.

Diagnosis:
If your doctor suspects Raynaud's, he or she will ask for a detailed description of your signs and symptoms. Your doctor will also examine you to rule out other medical problems that may cause similar signs and symptoms, such as a pinched nerve. Your doctor may perform an in-office cold-simulation test. This test may involve placing your hands in cool water or exposing you to cold air, to invoke an episode of Raynaud's.

Most doctors can diagnose Raynaud's directly from a description of your signs and symptoms. But it may be more difficult for them to identify whether you have Raynaud's alone or whether another disease, or some aspect of your lifestyle, is causing your symptoms.

To distinguish between primary and secondary Raynaud's, your doctor may perform an in-office test called nail fold capillaroscopy. During the test, the doctor examines your nail fold — the skin at the base of your fingernail — under a microscope. Tiny blood vessels (capillaries) near the nail fold that are enlarged or deformed may indicate an underlying disease. However, some secondary diseases can't be detected by this test.

If your doctor suspects that another condition, such as an autoimmune or connective tissue disease, underlies Raynaud's, he or she may order blood tests, such as :

  • Antinuclear antibodies (ANA) test. A positive test for the presence of these antibodies — produced by your immune system — indicates a stimulated immune system and is common in people who have connective tissue diseases or other autoimmune disorders.
  • Erythrocyte sedimentation rate (ESR). This blood test determines the rate at which red blood cells settle to the bottom of a tube in the space of an hour. A faster than normal rate may signal an underlying inflammatory or autoimmune disease. Autoimmune diseases are commonly associated with Raynaud's.

There's no single blood test to diagnose Raynaud's. Your doctor may order other tests, such as those that rule out diseases of the arteries, to help pinpoint a disease or condition that may be associated with Raynaud's.

Complications :
If Raynaud's is severe — which is rare — blood circulation to your fingers or toes could permanently diminish, causing deformities of your fingers or toes.

If an artery to an affected area becomes blocked completely, sores (skin ulcers) or dead tissue (gangrene) may develop. Ulcers and gangrene can be difficult to treat.

See your doctor right away if you have a history of severe Raynaud's and develop an ulcer or infection in one of your affected fingers or toes.

Treatment:
Self-care and preventive treatment usually are effective in alleviating mild symptoms of Raynaud's. If these aren't adequate, however, medications are available to treat more severe forms of the condition. The goals of treatment are to reduce the number and severity of attacks and to prevent tissue damage. Doctors also want to treat any underlying disease or condition.

Medications
Depending on the cause of your symptoms, medications may prove effective at treating Raynaud's. To widen (dilate) blood vessels and promote circulation, your doctor may prescribe:

  • Calcium channel blockers. These drugs relax and open up small blood vessels in your hands and feet. They decrease the frequency and severity of attacks in about two-thirds of people with Raynaud's. These drugs can also help heal skin ulcers on your fingers or toes. Examples include nifedipine (Adalat, Procardia), amlodipine (Norvasc), diltiazem (Cardizem, Dilacor), felodipine (Plendil), nisoldipine (Sular) and isradipine (DynaCirc Cr).
  • Alpha blockers. Some people find relief with drugs called alpha blockers, which counteract the actions of norepinephrine, a hormone that constricts blood vessels. Examples include prazosin (Minipress) and doxazosin (Cardura).
  • Vasodilators. Some doctors prescribe a vasodilator — a drug that relaxes blood vessels — such as nitroglycerin cream. You apply the cream to your fingers to help heal skin ulcers.

You and your doctor may find that one drug works better for you than another. Some drugs used to treat Raynaud's have side effects that may require you to stop taking the medication. A drug may also lose effectiveness over time. Work with your doctor to find what works best for you.

Some medications actually can aggravate Raynaud's by leading to increased blood vessel spasm. Your doctor may recommend that you avoid taking :

  • Certain over-the-counter (OTC) cold drugs. Examples include drugs that contain pseudoephedrine (Actifed, Chlor-Trimeton, Sudafed).
  • Beta blockers. This class of drug, used to treat high blood pressure and heart disease, may worsen Raynaud's. Examples include metoprolol (Lopressor, Toprol), nadolol (Corgard) and propranolol (Inderal).
  • Birth control pills. If you use birth control pills, you may wish to switch to another method of contraception because these drugs affect your circulation and may make you more prone to attacks.

If you have questions about how best to manage Raynaud's, contact your doctor. Your primary care doctor may refer you to a physician who specializes in treating Raynaud's.

Other treatments
Sometimes in cases of severe Raynaud's, approaches other than medications may be a treatment option :

  • Nerve surgery. Nerves called sympathetic nerves in your hands and feet control the opening and narrowing of blood vessels in your skin. Sometimes it's necessary in cases of severe Raynaud's to cut these nerves to interrupt their exaggerated response as seen in Raynaud's. Through small incisions in the affected hands or feet, a doctor strips away these tiny nerves around the blood vessels. The surgery, called sympathectomy, may reduce the frequency and duration of attacks, but it's not always successful.
  • Chemical injection. Doctors can inject chemicals to block sympathetic nerves in affected hands or feet. You may need to have the procedure repeated if symptoms return or persist.
  • Amputation. Sometimes, doctors need to amputate a finger or toe affected by Raynaud's in which the blood supply has been completely blocked and the tissue has developed gangrene. But this is rare.

Research
Scientists are evaluating the use of new drugs to treat Raynaud's. Some examples of drugs under study include the high blood pressure drug losartan (Cozaar) and a class of medication called prostaglandins.

One treatment that appears to have potential for Raynaud's is nitric oxide, which can be applied directly to the affected skin to provide relief during an attack or to prevent an attack. In studies, nitric oxide has been shown to improve blood circulation.

Prevention:
The most effective way to prevent tachycardias is to reduce your risk of developing heart disease. If you already have heart disease, monitor it and follow your treatment plan to lower your tachycardia risk.

Prevent heart disease
Raynaud's is a condition that you may need to manage for life once it develops. But there are ways to help prevent attacks :

  • Dress warmly outdoors. In winter, wear a hat, scarf, socks and boots, and mittens or gloves under mittens when you go outside. Put them on before you go outside. A hat is important because you lose a great deal of body heat through your head. Wear a coat with fairly snug cuffs to go around your mittens or gloves, to prevent cold air from reaching your hands. Wear earmuffs and a face mask if the tip of your nose and your earlobes are sensitive to cold. Run your car heater for a few minutes before driving in cold weather.
  • Take precautions indoors. Wear socks. When taking food out of the refrigerator or freezer, wear gloves, mittens or oven mitts. Some people find it helpful to wear mittens and socks to bed during winter. Because air conditioning can trigger attacks, turning down the air conditioning may help prevent attacks. You also may find it helpful to use insulated drinking glasses.
  • Consider moving to a location with a milder climate. Moving to a warmer climate may help people with severe Raynaud's. However, Raynaud's can occur even in warmer climates when the temperature decreases.
 
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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