Raynaud's disease

• Raynaud's disease is a condition that causes the blood vessels in the fingers, toes, nose, and ears to become constricted (narrowed) in response to cold temperatures or stress.
• During an episode, the affected areas of the body may feel numb or turn white in color. Symptoms will start to go away once the patient is exposed to warm temperatures or reduces his/her level of stress.
• When the condition occurs by itself, it is called Raynaud's disease (or primary Raynaud's). However, sometimes this disorder is a symptom of another medical condition, such as scleroderma (an autoimmune disease), lupus, or a blood vessel disease. In such cases, the disorder is called Raynaud's phenomenon (or secondary Raynaud's).
• Very few Americans have Raynaud's disease. It is more common among women than men. It is also more common in individuals who live in colder climates. Activities that put stress on the hands, such as playing the piano, typing, or frequently using vibrating tools (such as construction equipment), may increase an individual's risk of developing the disease.
• Individuals can prevent episodes of Raynaud's disease by dressing warmly and taking steps to reduce stress and anxiety. Treatment for Raynaud's disease may include medications to dilate (widen) the blood vessels. In more severe cases, surgery may be recommended.

• General: Raynaud's attacks are caused by exposure to cold temperatures or emotional stress or anxiety.
• Raynaud's disease (primary Raynaud's): The exact cause of Raynaud's disease, (also called primary Raynaud's) remains unknown. It has been suggested that genetics may play a role in the development of the disorder. Activities that put stress on the hands, such as playing the piano, typing, or frequently using vibrating tools (such as construction equipment), may increase an individual's risk of developing the disease.
• Raynaud's phenomenon (secondary Raynaud's): Raynaud's phenomenon, also called secondary Raynaud's, is a symptom of an underlying medical condition. For instance, Raynaud's phenomenon occurs in most people who have scleroderma, a rare autoimmune disorder and connective tissue disease that causes the skin to become thick and hard. Scleroderma occurs when a patient's immune system does not function properly. Normally, the immune system helps fight against diseases and infections. In scleroderma patients, the immune system attacks the person's own cells. Because scleroderma often causes the blood vessels in the hands and feet to become thick and constrict easily, it commonly causes Raynaud's phenomenon.
• In addition, Raynaud's phenomenon often occurs in patients with lupus, another autoimmune disease. Lupus affects many parts of the body, including the joints, skin, organs, and blood vessels. As a result, Raynaud's phenomenon often develops.
• Raynaud's phenomenon may be caused by another type of autoimmune disorder called rheumatoid arthritis. Unlike other types of arthritis, rheumatoid arthritis involves an overactive immune system. Rheumatoid arthritis is also considered an inflammatory condition because it causes pain, swelling, and joint stiffness.
• Other common medical conditions that may cause Raynaud's phenomenon include Sjogren's syndrome, diseases of the blood vessels (such as atherosclerosis or Buerger's disease, or primary pulmonary hypertension), carpal tunnel syndrome, underactive thyroid, prior injuries to the hands or feet (such as a broken wrist), and repetitive trauma that damages nerves serving blood vessels in the hands and feet.
• Smoking may also cause Raynaud's phenomenon. Smoking constricts the blood vessels, making individuals more susceptible to the condition.
• Some medications, including beta-blockers, birth control pills, chemotherapy drugs, medications that contain ergotamine, drugs that cause the blood vessels to narrow, and some over-the-counter cold medications, may contribute to the development of Raynaud's phenomenon. However, once the medication is stopped, symptoms will go away.
• A chemical used to make plastic, called vinyl chloride, may be associated with Raynaud's phenomenon. Some workers in the plastics industry who are exposed to this chemical have developed scleroderma, which often causes Raynaud's phenomenon.

• General: Symptoms of Raynaud's disease occur in response to cold temperatures or emotional stress or anxiety. The fingers and toes are most likely to be affected. However, other parts of the body, including the nose, ears, tongue, and cheeks, may also be affected. Symptoms depend on the frequency, duration, and severity of the blood vessel constrictions that cause this condition. An episode may last anywhere from less than one minute to several hours. Over time, episodes may become more severe. Once the affected body parts are warmed or stress is reduced, the symptoms will start to go away.
• Common symptoms: Cold temperatures or anxiety cause the skin to become white in color. The affected skin may then turn blue and feel cold and numb. The skin may become mildly swollen.
• The affected body parts may then start to feel numb, prickly, or sting as they become warmed or as stress is reduced. As circulation improves, the affected skin may turn red, throb, or swell.

• General: If Raynaud's disease is suspected, the doctor may perform a cold-stimulation test during a physical examination. During this test, the doctor may expose the patient to cold air or cool water to try to cause an episode of Raynaud's. Most doctors are able to make a diagnosis after observing the patient's symptoms in response to the test.
• Nail fold capillaroscopy: To distinguish between primary or secondary Raynaud's, a doctor may perform a nail fold capillaroscopy. During the test, the healthcare provider uses a microscope to look at the skin at the base of the fingernail. If the small blood vessels, called capillaries, are enlarged or deformed, it usually indicates that another health problem is causing symptoms of Raynaud's. However, some secondary diseases cannot be detected with this test. If the healthcare provider suspects that another medical condition, such as a connective disuse or autoimmune disease, is causing Raynaud's, a fluorescent antinuclear antibody (FANA) test or erythrocyte sedimentation rate (ESR) test may be recommended.
• Fluorescent antinuclear antibody (FANA) test: A blood test, called a fluorescent antinuclear antibody (FANA) test, may be performed if an autoimmune disorder is suspected. Raynaud's phenomenon is commonly associated with autoimmune disorders such as scleroderma or lupus. This test detects abnormal antibodies called autoantibodies. These autoantibodies bind to components of an individual's cells and cause the immune system to attack the body. If autoantibodies are present, this suggests that the patient has an autoimmune disorder. However, this test does not determine the specific type of autoimmune disorder that the patient has.
• Erythrocyte sedimentation rate (ESR): An erythrocyte sedimentation rate (ESR) test may be conducted to measure and monitor inflammation associated with autoimmune disorders. This blood test measures the rate in which red blood cells settle in unclotted blood.
• During an inflammatory response, the high proportion of fibrinogen in the blood causes red blood cells to stick together. The red blood cells form stacks called rouleaux, which settle faster than normal. Elevated levels usually occur in patients who have autoimmune disorders.
• An ESR test does not suggest a specific diagnosis. However, an increased rate may indicate an underlying inflammatory or autoimmune disease.

• General: Since it is rare for Raynaud's disease to become a severe condition, complications are uncommon. Patients who have severe Raynaud's disease should visit their doctors immediately if they develop ulcers or infections.
• Deformities: Severe Raynaud's disease may permanently diminish the blood supply to the fingers or toes. As a result, the fingers or toes may become disfigured.
• Hearing loss: Some cases of Raynaud's disease are associated with the frequent use of vibrating equipment, such as chain saws. In such cases, individuals may also have an increased risk of experiencing hearing loss as a result of the loud vibrating equipment. However, Raynaud's disease itself does not appear to affect hearing.
• Tissue damage and/or infection: In rare cases, a blood vessel may become completely blocked during an episode of Raynaud's disease. This may lead to sores, ulcers, or dead tissue (called gangrene). Patients who develop gangrene may need to have their affected fingers or toes amputated.

• General: Medications may be taken to widen, or dilate, the blood vessels, and increase blood circulation. However, the effectiveness of these drugs may lessen over time. Therefore, patients should stay in close contact with their doctors. In severe cases, other treatments, including nerve surgery, chemical injections, and amputation, may be recommended. However, Raynaud's disease is rarely a serious condition.
• Patients should tell their doctors if they are taking any other drugs (prescription or over-the-counter), herbs, or supplements because certain agents may worsen symptoms of Raynaud's disease.
• Calcium channel blockers: Medications called calcium channel blockers help open up the small blood vessels in the hands and feet. These drugs have been shown to reduce the frequency and severity of attacks in about 60% of patients with Raynaud's disease. They may also help heal skin ulcers on affected areas of skin. Commonly prescribed calcium channel blockers include nifedipine (Adalat® or Procardia®), amlodipine (Norvasc®), diltiazem (Cardizem® or Dilacor®), felodipine (Plendil®), nisoldipine (Sular®), and isradipine (DynaCirc Cr®).
• Alpha blockers: Patients may also benefit from alpha blockers such as prazosin (Minipress®) and doxazosin (Cardura®). These drugs reduce the action of norepinephrine, a hormone that causes blood vessels to become narrow.
• Vasodilators: Vasodilators, such as nitroglycerin cream, may also be recommended. The medication is applied to the skin to help heal skin ulcers.
• Nerve surgery: In severe cases a nerve surgery, called sympathectomy, may be recommended. The sympathetic nerves in the hands and feet control the widening and narrowing of the blood vessels in the skin. During surgery, these nerves are cut to prevent them from constricting and causing Raynaud's symptoms. Although this procedure has been shown to help reduce the frequency and duration of episodes, it is not always effective.
• Chemical injection: A healthcare provider may also inject chemicals into the skin to prevent the sympathetic nerves from constricting and causing symptoms. This procedure may need to be repeated if the symptoms continue or come back.
• Amputation: In very rare cases, Raynaud's disease may completely block blood supply to a finger and toe, causing the tissue to die (a condition called gangrene). In such cases, the affected finger or toe needs to be amputated.
• Medications to avoid: Some medications may worsen symptoms of Raynaud's disease. Therefore, patients should talk to their doctors before taking any new drugs (prescription or over-the-counter), herbs, or supplements. For instance, patients should avoid medications that contain pseudoephedrine (such as Actifed®, Chlor-Trimeton®, or Sudafed®), beta blockers, such as metoprolol (Lopressor® or Toprol®) or nadolol (Corgard®), and birth control pills.

C

Unclear or conflicting scientific evidence

• Acupuncture
  : Acupuncture is commonly used throughout the world. According to Chinese medicine theory, the human body contains a network of energy pathways through which vital energy, called "chi," circulates. These pathways contain specific points that function like gates, allowing chi to flow through the body. Needles are inserted into these points to regulate the flow of chi. There is currently insufficient evidence on the use of acupuncture in Raynaud's disease. Additional research is needed in this area.
• Needles must be sterile in order to avoid disease transmission. Avoid with valvular heart disease, medical conditions of unknown origin, or infections. Acupuncture should not be applied to the chest in patients with lung diseases or on any area that may rely on muscle tone to provide stability. Avoid use in infants, young children, or in patients with needle phobias. Use cautiously with bleeding disorders, neurological disorders, seizure disorders, or diabetes. Use cautiously in elderly or medically compromised patients. Use cautiously in patients who will drive or operate heavy machinery after acupuncture. Use cautiously if taking anticoagulants. Avoid if pregnant.
• Arginine
  : Early study in humans has looked at the effect of arginine on blood vessel activity in Raynaud's phenomenon. However, the effects of arginine are not clear. Large, well-designed trials are needed.
• Avoid with known allergy or hypersensitivity to arginine. Use cautiously with peripheral vascular disease, high blood pressure, alcohol-induced liver cirrhosis, or diabetes. Use cautiously in low birth weight infants and individuals on hemodialysis. Use cautiously if taking anticoagulants (blood thinners), beta-blockers, or calcium channel blockers. Avoid if pregnant or breastfeeding.
• Evening primrose oil
  : Evening primrose oil contains an omega-6 essential fatty acid called gamma-linolenic acid (GLA), which is believed to be the active ingredient. Not enough scientific information is available to advise the use of evening primrose oil for Raynaud's phenomenon at this time.
• Avoid if allergic to plants in the Onagraceae family (such as willow's herb or enchanter's nightshade), gamma-linolenic acid, or other ingredients in evening primrose oil. Use cautiously with seizure disorders. Use cautiously if taking phenothiazine neuroleptics. Stop use two weeks before surgery with anesthesia. Avoid if pregnant or breastfeeding due to a lack of safety evidence.
• Ginkgo biloba
  :
  Ginkgo biloba has been used medicinally for thousands of years. Results from limited available clinical study suggest that Ginkgo biloba may be effective in reducing the number of attacks in patients suffering from Raynaud's disease. In order to confirm these results, further clinical trials are required.
• Avoid if allergic or hypersensitive to members of the Ginkgoaceae
  family.
  If allergic to mango rind, sumac, poison ivy or oak or cashews, then allergy to ginkgo is possible. Avoid if taking anticoagulants due to an increased risk of bleeding. Ginkgo should be stopped two to three weeks before surgical procedures. Use cautiously with seizures or in children. Ginkgo seeds are dangerous and should be avoided. Skin irritation and itching may also occur due to ginkgo allergies. Do not use ginkgo in supplemental doses if pregnant or breastfeeding.

• Stay warm:
  Individuals with Raynaud's disease can prevent or reduce the risk of episodes by keeping their bodies warm. The most important areas to keep warm are the head (especially the ears and nose), fingers, and toes. Appropriate clothing, such as a hat, scarf, gloves/mittens, ear muffs, and socks should be worn in cold temperatures. When patients are inside, they should also take special precautions to stay warm. Socks should be worn inside. Make sure the home is warm enough in the colder months, and avoid turning the air conditioner on high during the warmer months. Using insulators on cold drinks may also be helpful. Some patients wear gloves or oven mitts when taking food out of the refrigerator or freezer.
• Limit the time spent in cold environments. Spend less time outdoors during the colder months. Individuals may also consider moving to regions that have warmer climates.
• Avoid smoking: Individuals are advised not to smoke. Smoking is a potential cause of Raynaud's disease because it constricts, or narrows, blood vessels. It may also worsen the condition in patients who already have Raynaud's disease.