Raynaud's syndrome

Definition

Raynaud's syndrome is a disorder in which the fingers or toes (digits) suddenly experience decreased blood circulation. It is also called Raynaud's disease.

Description

Raynaud's syndrome can be classified as one of two types: primary, or idiopathic (of unknown cause) disease; and secondary, which is also called Raynaud's phenomenon.

Primary Raynaud's disease is milder and causes fewer complications. About half of all cases of Raynaud's disease are of this type. Women are four times more likely than men to develop primary Raynaud's disease, and the average age of diagnosis is between 20 and 40 years. About 30% of all cases of primary Raynaud's disease progress after diagnosis, while 15% of cases actually improve.

Secondary Raynaud's disease is more complicated, severe, and more likely to get worse over time. A number of medical conditions or other triggers predispose a person to secondary Raynaud's disease, but do not directly cause the disorder. These include:

• Scleroderma. Scleroderma is a serious disease of the connective tissue, in which tissues of the skin, heart, esophagus, kidney, and lung become thickened, hard, and constricted. About 30% of patients diagnosed with scleroderma will then develop Raynaud's disease.
• Other diseases of connective tissue. These include systemic lupus erythematosus, rheumatoid arthritis , dermatomyositis, and polymyositis.
• Diseases that cause arterial blockage. These include atherosclerosis or hardening of the arteries.
• A severe form of high blood pressure which is caused by diseased arteries in the lung, called pulmonary hypertension.
• Disorders of the nervous system. These include herniated discs in the spine, strokes, tumors within the spinal cord, polio, and carpal tunnel syndrome.
• Other blood disorders.
• Trauma. Injuries that lead to Raynaud's are typically caused by exposure to constant vibration (workers who use chainsaws, jackhammers, or other vibrating equipment); repetitive movements (keyboard instrumentalists, assembly line workers, typists); electric shock; repeated use of the lower side of the palm as a hammer; or extreme cold (frostbite).
• Environmental toxins. Workers in the plastics industry who are exposed to high levels of vinyl chloride may develop a scleroderma-like illness that includes Raynaud's syndrome.
• Prescription medications. Drugs that increase the risk of developing Raynaud's include those used for migraine headaches or high blood pressure, and some cancer chemotherapy agents. Cases have also been reported of Raynaud's disease developing in reaction to quinine.

Causes & symptoms

Causes

Both primary and secondary types of Raynaud's symptoms are believed to be due to overreactive arterioles, or small arteries. While cold normally causes the muscle which makes up the walls of arteries to contract (squeeze down to become smaller), in Raynaud's disease the degree is extreme. Blood flow to the area is severely restricted. Some attacks may also be brought on or worsened by anxiety or emotional distress.

Although the cause of primary Raynaud's is not known as of 2002, researchers are focusing on

prostaglandin metabolism and the function of endothelial cells in the body. Prostaglandins are a group of unsaturated fatty acids involved in the contraction of smooth muscle and the control of inflammation and body temperature. Endothelial cells form the layer of smooth tissue that lines the inside of the heart, blood vessels, and other body cavities.

Recent advances in gene mapping and sequencing indicate that Raynaud's may be linked to abnormal forms of a gene known as the Fibrillin-1 gene. This gene affects the composition of the protein molecules in human connective tissue.

Symptoms

Classically, there are three distinct phases to an episode of Raynaud's symptoms. When first exposed to cold, the arteries respond by contracting intensely. The digits in question, or in rare instances, the tip of the nose or tongue, become pale and white as they are deprived of blood flow and the oxygen carried by the blood. In response, the veins and capillaries dilate, or expand. Because these vessels carry deoxygenated blood, the digit becomes cyanotic, which means that it turns blue. The digit often feels cold, numb, and tingly. After the digit begins to warm up again, the arteries dilate. Blood flow increases significantly, and the digits turn a bright red. During this phase, the patient often describes the digits as feeling warm, and throbbing painfully.

Raynaud's disease may initially only affect the tips of the fingers or toes. When the disease progresses, it may eventually affect the entire finger or toe. Ultimately, all the fingers or toes may be affected. About 10% of the time, a complication called sclerodactyly may occur. In sclerodactyly, the skin over the affected digits becomes tight, white, thick, smooth, and shiny.

When the most serious complications of Raynaud's disease or phenomenon occur, the affected digits develop deep sores, or ulcers, in the skin. The tissue may even die, thus becoming gangrenous, and requiring amputation. This complication occurs only about 1% of the time in primary Raynaud's disease.

Diagnosis

While the patient's symptoms will be the first clue pointing to Raynaud's disease, a number of tests may also be performed to confirm the diagnosis. Special blood tests called the antinuclear antibody test (ABA) and the erythrocyte sedimentation rate (ESR) are often abnormal when an individual has a connective tissue disease.

When a person has connective tissue disease, his or her capillaries are usually abnormal. A test called a nailfold capillary study can demonstrate such abnormalities. In this test, a drop of oil is placed on the skin at the base of the fingernail. This allows the capillaries in that area to be viewed more easily with a microscope.

A cold stimulation test may also be performed. In this test, specialized thermometers are taped to each of the digits that have experienced episodes of Raynaud's disease. The at-rest temperature of these digits is recorded. The hand or foot is then placed completely into a container of ice water for 20 seconds. After removing the hand or foot from this water, the temperature of the digits is recorded immediately. The temperature of the digits is recorded every five minutes until they reach the same temperature they were before being put into the ice water. A normal result occurs when this pretest temperature is reached in 15 minutes or less. If it takes more than 20 minutes, the test is considered suspicious for Raynaud's disease or phenomenon.

Treatment

The first type of treatment for Raynaud's symptoms is simple prevention. Patients need to stay warm, and keep hands and feet well covered in cold weather. Patients who smoke cigarettes should stop, because nicotine worsens the problem. Most people—especially those with primary Raynaud's—are able to deal with the disease by taking these basic measures.

Because episodes of Raynaud's disease have also been associated with stress and emotional upset, the disease may be improved by helping a patient learn to manage stress. Regular exercise is known to decrease stress and lower anxiety. Hypnosis, relaxation techniques, and visualization are also useful methods to help a patient gain control of his or her emotional responses. 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. Acupuncture is also used for treating these circulatory and heat distribution problems.

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. Several types of herbs have been suggested, including peony (Paeonia lactiflora ) and dong quai (Angelica sinensis ). The circulatory herbs cayenne (Capsicum frutescens ), ginger (Zingiber officinale ), and prickly ash (Zanthoxylum americanum ) can help enhance circulation to the extremities. Additionally, a tincture of one-half teaspoon of a combination of equal parts of ginkgo biloba, prickly ash, and ginger may be consumed three times daily.

Practitioners of traditional Chinese medicine (TCM) recommend certain formulas called si ni, which means cold extremities. TCM regards Raynaud's as an indication that the person is hypersensitive to cold outside the body because he or she is already cold inside. The Chinese practitioner will typically recommend various combinations of herbs regarded as warming to correct this condition. The si ni formulas contain different combinations of ginger, aconite , bupleurum, bitter orange, and honey-baked licorice .

Allopathic treatment

People with more severe cases of Raynaud's disease may need to be treated with medications to attempt to keep the arterioles relaxed and dilated. Some medications that are more commonly used to treat high blood pressure, such as calcium-channel blockers, or reserpine, are often effective for treatment of Raynaud's symptoms. Nitroglycerin paste can be used on the affected digits, and seems to be helpful in healing skin ulcers.

When a patient has secondary Raynaud's phenomenon, treatment of the coexisting condition may help control the Raynaud's as well. In the case of connective tissue disorders, this often involves treatment with corticosteroid medications.

Expected results

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 for serious complications (1%). In fact, about 50% of all patients do well by taking simple precautions, and never even require medications. The prognosis for people with secondary Raynaud's disease or phenomenon is less predictable. This prognosis depends greatly on the severity of the patient's other associated condition, such as scleroderma or lupus.

Prevention

As of 2002, there is no known way to prevent the development of Raynaud's disease. Once a person realizes that he or she suffers from this disorder, however, steps can be taken to reduce the frequency and severity of episodes.

Resources

BOOKS

Creager, Mark A., and Victor J. Dzau. "Vascular Disease of the Extremities." In Harrison's Principles of Internal Medicine, edited by Anthony S. Fauci, et al. 14th ed. New York: McGraw–Hill, 1998.

Pelletier, Kenneth R., MD. The Best Alternative Medicine. New York: Simon & Schuster, 2002.

"Raynaud's Disease and Phenomenon," Section 16, Chapter 212 in The Merck Manual of Diagnosis and Therapy, 17th edition, edited by Mark H. Beers, MD, and Robert Berkow, MD. Whitehouse Station, NJ: Merck Research Laboratories, 2003.

PERIODICALS

Agarwal, N., and B. Cherascu. "Concomitant Acral Necrosis and Haemolytic Uraemic Syndrome Following Ingestion of Quinine." Journal of Postgraduate Medicine 48 (July-September 2002): 197-198.

Dharmananda, Subhuti. "Raynaud's Disease: Chinese Medical Perspective." Internet Journal of the Institute for Traditional Medicine and Preventive Health Care 1 (July 2002): 1-7.

Heitmann, C., M. Pelzer, M. Trankle, et al. "The Hypothenar Hammer Syndrome." [in German] Der Unfallchirurg 105 (September 2002): 833-836.

Kodera, T., F. K. Tan, T. Sasaki, et al. "Association of 5'-Untranslated Region of the Fibrillin-1 Gene with Japanese Scleroderma." Gene 297 (September 4, 2002): 61-67.

ORGANIZATIONS

American College of Rheumatology. 1800 Century Place, Suite 250, Atlanta, GA 30345. (404) 633-3777. <www.rheumatology.org>.

Institute of Traditional Medicine. 2017 SE Hawthorne Blvd., Portland, OR 97214. (503) 233-4907. <www.itmonline.org>.

National Institute of Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse. National Institutes of Health, 1 AMS Circle, Bethesda, MD 20892. (301) 495-4484 or (toll-free)(877) 22-NIAMS. <www.niams.nih.gov>.

OTHER

National Institutes of Health. Questions and Answers About Raynaud's Phenomenon. Bethesda, MD: National Institutes of Health, 2001. NIH Publication No. 01-4911.

"Raynaud's Disease." http://www.alternaticemedicine.com/digest/issue04/04354R00.sh.

Kathleen D. Wright

Rebecca J. Frey, PhD