Raynaud's disease refers to a disorder in which the fingers or toes (digits) suddenly experience decreased blood circulation. It is characterized by repeated episodes of color changes of the skin of digits on cold exposure or emotional stress.
Raynaud's disease can be classified as one of two types: primary (or idiopathic) and secondary (also called Raynaud's phenomenon). Primary Raynaud's disease has no predisposing factor, is more mild, and causes fewer complications. About half of all cases of Raynaud's disease are of this type. Women are five times more likely than men to develop primary Raynaud's disease. The average age of diagnosis is between 20 and 40 years. Approximately three out of ten people with primary Raynaud's disease eventually progress to secondary Raynaud's disease after diagnosis. About 15% of individuals improve.
Secondary Raynaud's disease is the same as primary Raynaud's disease, but occurs in individuals with a predisposing factor, usually a form of collagen vascular disease. What is typically identified as primary Raynaud's is
later identified as secondary once a predisposing disease is diagnosed. This occurs in approximately 30% of patients. As a result, the secondary type is often more complicated and severe, and is more likely to worsen.
Several related conditions that predispose persons to secondary Raynaud's disease include scleroderma, systemetic lupus erythematosus, rheumatoid arthritis and polymyositis. Pulmonary hypertension and some nervous system disorders such as herniated discs and tumors within the spinal column, strokes, and polio can progress to Raynaud's disease. Finally, injuries due to mechanical trauma caused by vibration (such as that associated with chain saws and jackhammers), repetitive motion (carpal tunnel syndrome), electrical shock, and exposure to extreme cold can led to the development of Raynaud's disease. Some drugs used to control high blood pressure or migraine headaches have been known to cause Raynaud's disease.
The prevalence of Reynaud's Phenomena in the general population varies from 4–15%. Females are seven times more likely to develop Raynaud's diseases than are men. The problem has not been correlated with coffee consumption, dietary habits, occupational history (excepting exposure to vibration) and exposure to most drugs. An association between Raynaud's disease and migraine headaches and has been reported. Secondary Raynaud's disease is common among individuals with systemic lupus erythematosus in tropical countries.
Causes and symptoms
There is significant familial aggregation of primary Raynaud's disease. However, as of 2001, no causative gene has been identified.
Risk factors for Raynaud's disease differ between males and females. Age and smoking seem to be associated with Raynaud's disease only in men, while the associations of marital status and alcohol use with Raynaud's disease are usually only observed in women. These findings suggest that different mechanisms influence the expression of Raynaud's disease in men and women.
Both primary and secondary Raynaud's disease signs and symptoms are thought to be due to arterioles over-reacting to stimuli. Cold normally causes the tiny muscles in the walls of arteries to contract, thus reducing the amount of blood that can flow through them. In people with Raynaud's disease, the extent of constriction is extreme, thus severely restricting blood flow. Attacks or their effects may be brought on or worsened by anxiety or emotional distress.
There are three distinct phases to an episode of Raynaud's disease. When first exposed to cold, small arteries respond with intense contractions (vasoconstriction). The affected fingers or toes (in rare instances, the tip of the nose or tongue) become pale and white because they are deprived of blood and, thus, oxygen. In response, capillaries and veins expand (dilate). Because these vessels are carrying deoxygenated blood, the affected area then becomes blue in color. The area often feels cold and tingly or numb. After the area begins to warm up, the arteries dilate. Blood flow is significantly increased. This changes the color of the area to a bright red. During this phase, persons often describe the affected area as feeling warm and throbbing painfully.
Raynaud's disease may initially affect only the tips of fingers or toes. As the disease progresses, it may eventually involve all of one or two digits. Ultimately, all the fingers or toes may be affected. About one person in ten, will experience a complication called sclerodactyly. In sclerodactyly, the skin over the involved digits becomes tight, white, thick, smooth and shiny. In approximately 1% of cases of Raynaud's disease, deep sores (ulcers) may develop in the skin. In rare cases of frequent, repetitive bouts of severe ischemia (decreased supply of oxygenated blood to tissues or organs), tissue loss, or gangrene may result and amputation may be required.
Primary Raynaud's disease is diagnosed following the Allen Brown criteria. There are four components. The certainty of the diagnosis and severity of the disease increase as more criteria are met. The first is that at least two of the three color changes must occur during attacks provoked by cold and or stress. The second is that episodes must periodically occur for at least two years. The third is that attacks must occur in both the hands and the feet in the absence of vascular occlusive disease. The last is that there is no other identifiable cause for the Raynaud's episodes.
A cold stimulation test may also be performed to help to confirm a diagnosis of Raynaud's disease. The temperature of affected fingers or toes is taken. The hand or foot is then placed completely into a container of ice water for 20 seconds. After removal from the water, the temperature of the affected digits is immediately recorded. The temperature is retaken every five minutes until it returns to the pre-immersion level. Most individuals recover normal temperature within 15 minutes. People with Raynaud's disease may require 20 minutes or more to reach their pre-immersion temperature.
Laboratory testing is performed frequently. However, these results are often inconclusive for several reasons. Provocative testing such as the ice emergence just described, is difficult to interpret because there is considerable overlap between normal and abnormal results. The antinuclear antibody test of blood is usually negative in Raynaud's disease. Capillary beds under finger nails usually appear normal. Erythrocyte sedimentation rates are often abnormal in people with connective tissue diseases. Unfortunately, this finding is not consistent in people with Raynaud's disease.
There is no known way to prevent the development of Raynaud's disease. Further, there is no known cure for this condition. Therefore, avoidance of the trigger is the best supportive management available. Most cases of primary Raynaud's disease can be controlled with proper medical care and avoidance.
Many people are able to find relief by simply adjusting their lifestyles. Affected individuals need to stay warm, and keep their hands and feet well covered in cold weather. Layered clothing, scarves, heavy coats, heavy socks, and mittens under gloves are suggested because gloves alone allow heat to escape. It is also recommended that patients cover or close the space between their sleeves and mittens. Indoors, they should wear socks and comfortable shoes. Smokers should quit as nicotine will worsen the problem. Avoid the use of vibrating tools as well.
People with severe cases of Raynaud's disease may need to be treated with medications to help keep the arterioles relaxed and dilated. Medications such as calcium-channel blockers, reserpine or nitroglycerin may be prescribed to relax artery walls and improve blood flow.
Because episodes of Raynaud's disease have also been associated with stress and emotional upset, the condition
Biofeedback training is a technique during which a patient is given continuous information on the temperature of his or her digits, and then taught to voluntarily control this temperature. Some alternative practitioners believe that certain dietary supplements and herbs may be helpful in decreasing the vessel spasm of Raynaud's disease. Suggested supplements include vitamin E (found in fruits, vegetables, seeds, and nuts), magnesium (found in seeds, nuts, fish, beans, and dark green vegetables), and fish oils. The circulatory herbs cayenne, ginger and prickly ash may help enhance circulation to affected areas.
The prognosis for most people with Raynaud's disease is very good. In general, primary Raynaud's disease has the best prognosis, with a relatively small chance (1%) of serious complications. Approximately half of all affected individuals do well by taking simple precautions, and never require medication. The prognosis for people with secondary Raynaud's disease (or phenomenon) is less predictable. This prognosis depends greatly on the severity of other associated conditions such as scleroderma, lupus, or Sjögren syndrome.
There is no way to prevent the development of Raynaud's disease. Once an individual realizes that he or she suffers from this disorder, however, steps can be taken to reduce the frequency and severity of episodes.
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American Heart Association. 7272 Greenville Ave., Dallas, TX 75231-4596. (214) 373-6300 or (800) 242-8721. <firstname.lastname@example.org.> <http://www.americanheart.org>.
Irish Raynaud's and Scleroderma Society. PO Box 2958 Foxrock, Dublin 18, Ireland. (01) 235 0900. <email@example.com>.
National Heart, Lung, and Blood Institute. PO Box 30105, Bethesda, MD 20824-0105. (301) 592-8573. <firstname.lastname@example.org.> <http://www.nhlbi.nih.gov>.
National Organization for Rare Disorders (NORD). PO Box 8923, New Fairfield, CT 06812-8923. (203) 746-6518 or (800) 999-6673. Fax: (203) 746-6481. <http://www.rarediseases.org>.
Raynaud's & Scleroderma Association (UK). 112 Crewe Road, Alsager, Cheshire, ST7 2JA. UK (44) (0) 1270 872776. <email@example.com>. <http://www.raynauds.demon.co.uk>.
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L. Fleming Fallon, Jr., MD, PhD, DrPH
Arteriole—The smallest type of artery.
Artery—A blood vessel that carries blood away from the heart to peripheral tissues.
Gangrene—Death of a tissue, usually caused by insufficient blood supply and followed by bacterial infection of the tissue.
Idiopathic—Of unknown origin.
Polymyositis—An inflammation of many muscles.
Pulmonary hypertension—A severe form of high blood pressure caused by diseased arteries in the lung.
Rheumatoid arthritis—Chronic, autoimmune disease marked by inflammation of the membranes surrounding joints.
Scleroderma—A relatively rare autoimmune disease affecting blood vessels and connective tissue that makes skin appear thickened.
Systemoc lupus erythematosus—A chronic inflammatory disease that affects many tissues and parts of the body including the skin.