Raynaud's Phenomenon Diagnosis

  • Diagnosis

    Raynaud's phenomenon (RP) is characterized by episodic constriction of the small arteries. The phenomenon is an exaggerated response to cold or other triggers, and eventually results in reduced blood flow to certain areas of the body. The disorder most commonly affects the fingers and toes, the ears, and the tip of the nose, but may affect other areas as well. When Raynaud's phenomenon (RP) occurs as an isolated symptom, it is known as primary RP, or Raynaud's phenomenon, and tends to be harmless. In contrast, when RP occurs as a symptom of an underlying illness (for example, systemic lupus erythematosus), it is known as secondary RP, and is often more severe and more difficult to treat. While there is no cure for RP, symptoms can be controlled, and complications can be prevented. The severity of RP varies widely from patient to patient.

    RP may affect from 5% to 10% of the U.S. population. RP is more common in women than in men. Raynaud's phenomenon is far more prevalent than secondary RP, and most often affects young women between the ages of 15 and 40. The incidence of RP is higher in colder climates and varies among ethnic groups.

    Episodes are often triggered by cold or stress. When exposed to cold, the body's normal response is to constrict blood vessels (a process known as vasoconstriction) in the extremities, thereby shunting blood towards deeper vessels. This response slows heat loss, and maintains the body's core temperature.

    In patients with RP, vasoconstriction is inappropriately exaggerated, and causes the same small arteries to close spasmodically. This is known as vasospasm, and drastically reduces blood flow to tissues supplied by these arteries. The affected tissues may then become starved for oxygen and other nutrients.

    While secondary RP is seen in many other diseases, not everyone with those diseases develops RP. Some of the diseases in which secondary RP can occur include the following:

    • Rheumatoid arthritis
    • Systemic lupus erythematosus
    • Systemic sclerosis
    • Sjögren's syndrome
    • Dermatomyositis and polymyositis
    • Systemic vasculitides
    • Carpal tunnel syndrome and other nerve entrapment syndromes
    • Atherosclerosis
    • Thromboangiitis obliterans (Buerger's disease)
    • Polycythemia

    Certain drugs and toxins have been linked to RP. Some of the more commonly implicated agents include:

    • Beta-blockers
    • Ergot alkaloids
    • Certain cancer chemotherapy agents (particularly bleomycin)
    • Vinyl chloride
    • Pseudoephedrine
    • Phenylpropanolamine (now discontinued by the Food and Drug Administration)
    • Cocaine
    • Caffeine
    • Nicotine

    As a result of reduced blood flow, the skin may turn from normal-colored to white to blue to red. Sensory symptoms may be absent, or where present, may range from mild numbness and tingling to severe pain. In the most severe cases, skin ulcerations, infections, and permanent tissue loss may occur Figure 01 Figure 02. In the early stages of RP, the affected areas will become pale and cold as circulation diminishes. If the blood flow does not return, the affected areas turn blue and become increasingly painful as the tissue becomes oxygen-poor. Acute attacks can last from seconds to hours. As the arteries relax and blood flow is reestablished, throbbing pain and redness may occur. Individual patients may experience some or all of these changes.

    With severe or repeated attacks, skin ulcerations may develop. If left untreated, the ulcers may become infected and may lead to permanent tissue damage and loss.

    Click to enlarge: Fingertip ulcers

    Figure 01. Fingertip ulcers

    Click to enlarge: White fingertips

    Figure 02. White fingertips

    Young women between 15 to 40 years of age are at particular risk for developing Raynaud's phenomenon. The disease often begins in the teenage years. Many cases of Raynaud's phenomenon develop initially in women of childbearing age. The reasons for this predisposition are not known.

    People whose fingers are exposed to repeated traumatic stress may be more vulnerable to RP. People who experience repeated trauma to the fingers (such as typists, pianists, or jackhammer operators) appear to have a greater risk for developing RP. People with previous histories of frostbite are also at increased risk.

    Your physician will assess the nature and severity of your symptoms. The physician will then determine whether there is an underlying illness by taking a detailed medical history and performing a thorough general physical examination. Particular attention may be given to skin or joint abnormalities.

    Your physician may perform more specialized diagnostic tests. Cold stimulation test. A sensor is first attached to the fingers to measure baseline temperature. The fingers will then be chilled, either with cold water or with another cooling device. After a few minutes, cold stimulation will be discontinued, and finger skin temperature will be measured every five minutes until it returns to baseline. Skin temperature returns to baseline within 15 minutes in people without RP. If it takes more than 20 minutes for skin temperature to recover, RP is highly suspected. Similar testing can also be performed using ultrasound measurements of blood flow before and after cold provocation.

    Nailfold capillaroscopy. This test involves examining the capillaries at the base of the fingernails (nailfolds) under magnification for abnormalities. Nailfold capillary abnormalities suggest underlying disease.

    Tendon friction rub. The physician may examine areas overlying tendons to detect unusual signs of tissue irritation that may reflect underlying illnesses.

    Antinuclear antibody (ANA) test. ANAs are abnormal antibodies that can be detected with a simple blood test. These antibodies are commonly found in autoimmune diseases—disorders in which the immune system mistakenly attacks normal tissue.

    Take steps to prevent attacks by keeping yourself warm. When indoors, maintain a warm temperature, and avoid drafts, direct exposure to fans, or currents of air-conditioning.

    Wear oven mitts when taking food out of the refrigerator or freezer. Wear appropriately insulated clothing at all times; mittens or fur-lined gloves in cold weather can be used with portable hand warmers inserted within. Drinking something warm can help before venturing into cold weather.

    Avoid medicines and other substances (notably caffeine and nicotine) known to precipitate attacks, and make sure to drink enough fluids.

    Avoid injuring areas affected by RP, as the reduced blood flow may prevent scrapes and cuts from healing efficiently.

    As emotional stress can cause blood vessels to constrict, make sure to address psychological issues relating to or independent of medical illness. Relaxation techniques, psychotherapeutic approaches, and stress modification can help.

    Some patients with primary RP appear to respond to biofeedback, which is a technique whereby patients are trained to increased finger temperature in response to reinforcing signals. However, patients with secondary RP do not respond as well to biofeedback as those with primary RP.

    Conditioning exercises also help some patients. In this approach, the hands are repeatedly exposed to warm water while the rest of the body is exposed to cold temperatures. Over time, as the body begins to associate being cold with having warm hands, feeling cold may elicit a warm-hand response. Conditioning appears to help only patients with primary RP, and usually requires "reconditioning" to maintain the warm-hand reaction.

  • Prevention and Screening

    Take steps to prevent attacks by keeping yourself warm. When indoors, maintain a warm temperature, and avoid drafts, direct exposure to fans, or currents of air-conditioning.

    Wear oven mitts when taking food out of the refrigerator or freezer. Wear appropriately insulated clothing at all times; mittens or fur-lined gloves in cold weather can be used with portable hand warmers inserted within. Drinking something warm can help before venturing into cold weather.

    Avoid medicines and other substances (notably caffeine and nicotine) known to precipitate attacks, and make sure to drink enough fluids.

    Avoid injuring areas affected by RP, as the reduced blood flow may prevent scrapes and cuts from healing efficiently.

    As emotional stress can cause blood vessels to constrict, make sure to address psychological issues relating to or independent of medical illness. Relaxation techniques, psychotherapeutic approaches, and stress modification can help.

    Some patients with primary RP appear to respond to biofeedback, which is a technique whereby patients are trained to increased finger temperature in response to reinforcing signals. However, patients with secondary RP do not respond as well to biofeedback as those with primary RP.

    Conditioning exercises also help some patients. In this approach, the hands are repeatedly exposed to warm water while the rest of the body is exposed to cold temperatures. Over time, as the body begins to associate being cold with having warm hands, feeling cold may elicit a warm-hand response. Conditioning appears to help only patients with primary RP, and usually requires "reconditioning" to maintain the warm-hand reaction.

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