Raynaud disease

Definition

Raynaud 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 during cold exposure or emotional stress.

Description

Raynaud disease can be classified as one of two types: primary (or idiopathic) and secondary (also called Raynaud phenomenon). Primary Raynaud disease has no predisposing factor, is more mild, and causes fewer complications. About half of all cases of Raynaud disease are of this type. Women are five times more likely than men to develop primary Raynaud disease. The average age of diagnosis is between 20 and 40 years. Approximately three out of ten people with primary Raynaud disease eventually progress to secondary Raynaud disease after diagnosis. About 15% of individuals improve.

Secondary Raynaud disease is the same as primary Raynaud disease, but occurs in individuals with a predisposing factor, usually a form of collagen vascular disease. What is typically identified as primary Raynaud may be later identified as secondary once a predisposing disease is diagnosed. This occurs in approximately 30% of patients. As a result of the predisposing disease, the secondary type is often more complicated and severe, and is more likely to worsen.

Several related conditions that predispose persons to secondary Raynaud disease include scleroderma , 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 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 disease. Some drugs used to control high blood pressure or migraine headaches have been known to cause Raynaud disease.

Genetic profile

There is significant familial aggregation of primary Raynaud disease. However, no causative gene has been identified.

Risk factors for Raynaud disease differ between males and females. Age and smoking seem to be associated with Raynaud disease only in men, while the associations of marital status and alcohol use with Raynaud disease are usually only observed in women. These findings suggest that different mechanisms influence the expression of Raynaud disease in men and women.

Demographics

The prevalence of Raynaud phenomena in the general population varies from 4–15%. Females are seven times more likely to develop Raynaud diseases than are men. The problem has not been correlated with coffee consumption, dietary habits, occupational history (excepting exposure to vibration), or exposure to most drugs. An association between Raynaud disease and migraine headaches has been reported. Secondary Raynaud disease is common among individuals with systemic lupus erythematosus in tropical countries.

Signs and symptoms

Both primary and secondary Raynaud 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 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 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 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 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.

Diagnosis

Primary Raynaud 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 occur periodically 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 episodes.

A cold stimulation test may also be performed to help to confirm a diagnosis of Raynaud 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 taken every five minutes until it returns to the pre-immersion level. Most individuals recover normal temperature within 15 minutes. People with Raynaud 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 immersion 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 disease. Capillary beds under fingernails 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 disease.

Treatment and management

There is no known way to prevent the development of Raynaud 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 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 over 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. Excessive emotional stress should be avoided. Smokers should quit as nicotine worsens the problem. The use of vibrating tools should be avoided as well.

Biofeedback has been used with some success in treating primary Raynaud. This involves teaching people

to "think" their fingers and toes to be warm by willing blood to flow through affected arterioles. Biofeedback has had only limited success. Occasionally, medications such as calcium-channel blockers, reserpine or nitroglycerin may be prescribed to relax artery walls and improve blood flow.

Because episodes of Raynaud disease have also been associated with stress and emotional upset, the condition may be improved by learning to manage stress. Regular exercise is known to decrease stress and lower anxiety. Hypnosis, relaxation techniques, and visualization are also useful methods to help control emotions.

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 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.

Prognosis

The prognosis for most people with Raynaud disease is very good. In general, primary Raynaud 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 disease (or phenomenon) is less predictable. This prognosis depends greatly on the severity of other associated conditions such as scleroderma, lupus, or Sjögren syndrome.

Resources

BOOKS

Coffman, Jay D. Raynaud Phenomenon. New York: Oxford University Press, 1989.

Fauci, Anthony S. "The Vasculitis Syndromes." In Harrison's Principles of Internal Medicine, edited by Anthony Fauci et al., 14th ed. New York: McGraw Hill, 1998, 1910-1922.

Rosenwasser, Lanny J. "The Vasaculitic Syndromes." In Cecil Textbook of Medicine, edited by Lee Goldman, et al. 21st ed. Philadelphia: Saunders, 2000, pp. 1524-1527.

PERIODICALS

Brand, F. N., M. G. Larson, W. B. Kannel, and J. M. McGuirk. "The occurrence of Raynaud phenomenon in a general population: the Framingham Study." Vascular Medicine 2, no. 4 (November 1997): 296-301.

Fraenkel, L., et al. "Different factors influencing the expression of Raynaud phenomenon in men and women." Arthritis and Rheumatology 42, no. 2 (February 1999): 306-310.

Voulgari, P. V., et al. "Prevalence of Raynaud phenomenon in a healthy Greek population." Annals of Rheumatic Disease 59, no. 3 (March 2000): 206-210.

ORGANIZATIONS

American Heart Association. 7272 Greenville Ave., Dallas, TX 75231-4596. (214) 373-6300 or (800) 242-8721. [email protected]. <http://www.americanheart.org>.

Irish Raynaud and Scleroderma Society. PO Box 2958 Foxrock, Dublin 18, Ireland. (01) 235 0900. [email protected].

National Heart, Lung, and Blood Institute. PO Box 30105, Bethesda, MD 20824-0105. (301) 592-8573. [email protected]. <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 & Scleroderma Association (UK). 112 Crewe Road, Alsager, Cheshire, ST7 2JA, UK. (44) (0) 1270 872776. [email protected]. <http://www.raynauds.demon.co.uk>.

WEBSITES

Arthritis Foundation. Dublin, Ireland. <http//www.arthritisfoundation.com/>.

British Sjögren's Syndrome Association. <http://ourworld.copmpuserve.com/homepages/BSSAssociation>.

Raynaud & Scleroderma Association. United Kingdom. <http://www.Raunaud's.demon.co.uk/>.

Rodriguez, J., and S. Wasson. "Raynaud Disease." Wayne State University School of Medicine. <http://www.med.wayne.edu/raynauds/>.

L. Fleming Fallon, Jr., MD, PhD, DrPH