Raynaud's phenomenon is a disorder that affects the blood vessels in the fingers, toes, ears, and nose. This disorder is characterized by episodic attacks, called vasospastic attacks, that cause the blood vessels in the digits (fingers and toes) to constrict (narrow). Although estimates vary, recent surveys show that Raynaud's phenomenon may affect 5 to 10 percent of the general population in the United States. Women are more likely than men to have the disorder. Raynaud's phenomenon appears to be more common in people who live in colder climates. However, people with the disorder who live in milder climates may have more attacks during periods of colder weather.
For most people, an attack is usually triggered by exposure to cold or emotional stress. In general, attacks affect the fingers or toes but may affect the nose, lips, or ear lobes.
Doctors classify Raynaud's phenomenon as either the primary or the secondary form. In medical literature, "primary Raynaud's phenomenon" may also be called Raynaud's disease, idiopathic Raynaud's phenomenon, or primary Raynaud's syndrome. The terms idiopathic and primary both mean that the cause is unknown.
Primary Raynaud's Phenomenon: Most people who have Raynaud's phenomenon have the primary form (the milder version). A person who has primary Raynaud's phenomenon has no underlying disease or associated medical problems. More women than men are affected, and approximately 75 percent of all cases are diagnosed in women who are between 15 and 40 years old.
People who have only vasospastic attacks for several years, without involvement of other body systems or organs, rarely have or will develop a secondary disease (that is, a connective tissue disorder such as scleroderma) later. Several researchers who studied people who appeared to have primary Raynaud's phenomenon over long periods of time found that less than 9 percent of these people developed a secondary disease.
Secondary Raynaud's Phenomenon: Although secondary Raynaud's phenomenon is much less common than the primary form, it is often a more complex and serious disorder. Secondary means that patients have an underlying disease or condition that causes Raynaud's phenomenon. Connective tissue diseases are the most common cause of secondary Raynaud's phenomenon. Some of these diseases reduce blood flow to the digits by causing blood vessel walls to thicken and the vessels to constrict too easily. Raynaud's phenomenon is seen in approximately 85 to 95 percent of patients with scleroderma and mixed connective tissue disease, and it is present in about one-third of patients with systemic lupus erythematosus. For most people with lupus, Raynaud's phenomenon acts like the primary form of the disorder. Raynaud's phenomenon also can occur in patients who have other connective tissue diseases, including Sjögren's syndrome, dermatomyositis, and polymyositis.
Possible causes of secondary Raynaud's phenomenon, other than connective tissue diseases, are carpal tunnel syndrome and obstructive arterial disease (blood vessel disease). Some drugs, including beta-blockers (used to treat high blood pressure), ergotamine preparations (used for migraine headaches), certain agents used in cancer chemotherapy, and drugs that cause vasoconstriction such as some over-the-counter cold medications and narcotics are linked to Raynaud's phenomenon.
People in certain occupations may be more vulnerable to secondary Raynaud's phenomenon. Some workers in the plastics industry (who are exposed to vinyl chloride) develop a scleroderma-like illness, of which Raynaud's phenomenon can be a part. Workers who operate vibrating tools can develop a type of Raynaud's phenomenon called vibration-induced white finger. In addition, people whose fingers are subject to repeated stress, such as typing or playing the piano, are more vulnerable to the disorder.
People with secondary Raynaud's phenomenon often experience associated medical problems. The more serious problems are skin ulcers (sores) or gangrene (tissue death) in the fingers or toes. Painful ulcers and gangrene are fairly common and can be difficult to treat. In addition, a person may experience heartburn or difficulty in swallowing. These two problems are caused by weakness in the muscle of the esophagus (the tube that takes food and liquids from the mouth to the stomach) that can occur in people with connective tissue diseases.
If a doctor suspects Raynaud's phenomenon, he or she will ask the patient for a detailed medical history. The doctor will then examine the patient to rule out other medical problems. The patient might have a vasospastic attack during the office visit, which makes it easier for the doctor to diagnose Raynaud's phenomenon. Most doctors find it fairly easy to diagnose Raynaud's phenomenon but more difficult to identify the form of the disorder. (See below for the criteria doctors use to diagnose primary or secondary Raynaud's phenomenon.)
Nailfold capillaroscopy (study of capillaries under a microscope) can help the doctor distinguish between primary and secondary Raynaud's phenomenon. During this test, the doctor puts a drop of oil on the patient's nailfolds, the skin at the base of the fingernail. The doctor then examines the nailfolds under a microscope to look for abnormalities of the tiny blood vessels called capillaries. If the capillaries are enlarged or deformed, the patient may have a connective tissue disease.
The doctor may also order two particular blood tests, an antinuclear antibody test (ANA) and an erythrocyte sedimentation rate (ESR). The ANA test determines whether the body is producing special proteins (antibodies) often found in people who have connective tissue diseases or other autoimmune disorders. The ESR test is a measure of inflammation in the body and tests how fast red blood cells settle out of unclotted blood. Inflammation in the body causes an elevated ESR.
The aims of treatment are to reduce the number and severity of attacks and to prevent tissue damage and loss in the fingers and toes. Most doctors are conservative in treating patients with primary and secondary Raynaud's phenomenon; that is, they recommend nondrug treatments and self-help measures first. Doctors may prescribe medications for some patients, usually those with secondary Raynaud's phenomenon. In addition, patients are treated for any underlying disease or condition that causes secondary Raynaud's phenomenon.
People with Raynaud's phenomenon should also be aware that air conditioning can trigger attacks. Turning down the air conditioning or wearing a sweater may help prevent attacks. Some people find it helpful to use insulated drinking glasses and to put on gloves before handling frozen or refrigerated foods.
People with secondary Raynaud's phenomenon are more likely than those with the primary form to be treated with medications. Many doctors believe that the most effective and safest drugs are calcium-channel blockers, which relax smooth muscle and dilate the small blood vessels. These drugs decrease the frequency and severity of attacks in about two-thirds of patients who have primary and secondary Raynaud's phenomenon. These drugs also can help heal skin ulcers on the fingers or toes.
Other patients have found relief with drugs called alpha-blockers that counteract the actions of norepinephrine, a hormone that constricts blood vessels. Some doctors prescribe a nonspecific vasodilator (drug that relaxes blood vessels), such as nitroglycerine paste, which is applied to the fingers, to help heal skin ulcers. Patients should keep in mind that the treatment for Raynaud's phenomenon is not always successful. Often, patients with the secondary form will not respond as well to treatment as those with the primary form of the disorder.
Patients may find that one drug works better than another. Some people may experience side effects that require stopping the medication. For other people, a drug may become less effective over time. Women of childbearing age should know that the medications used to treat Raynaud's phenomenon may affect the growing fetus. Therefore, women who are pregnant or are trying to become pregnant should avoid taking these medications if possible.
Researchers are studying the use of other drugs to treat Raynaud's phenomenon; for example, oral and intravenous prostaglandins, such as iloprost. Other investigators are studying the molecular mechanisms behind Raynaud's phenomenon and the anatomy of blood vessels. Several medical centers in the United States are studying the use of biofeedback to control attacks. Researchers studying scleroderma and other connective tissue diseases are also investigating Raynaud's phenomenon in relation to these diseases.
This is the main voluntary organization devoted to all forms of arthritis. The Foundation publishes a free pamphlet on Raynaud's phenomenon and also provides physician referrals.
The NIAMS gratefully acknowledges the assistance of Phillip J. Clements, M.D., of the University of California, Los Angeles; Jay D. Coffman, M.D., of the Boston University Medical Center; and Frederick M. Wigley, M.D., of The Johns Hopkins University School of Medicine in the preparation and review of this fact sheet.
The National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse (NAMSIC) is a public service sponsored by the NIAMS that provides health information and information sources. The NIAMS, a part of the National Institutes of Health (NIH), leads the Federal medical research effort in arthritis and musculoskeletal and skin diseases. The NIAMS sponsors research and research training throughout the United States as well as on the NIH campus in Bethesda, MD, and disseminates health and research information.
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