An Overview of Raynaud's Syndrome

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Raynaud's syndrome is a condition in which a person's fingers, and sometimes toes, turn blue and/or white upon exposure to cold, and then bright red upon rewarming. These symptoms— caused by the constriction of small blood vessels in the hands or feet—can last for seconds to hours, but most often last around 15 minutes. There are two types: Primary Raynaud's syndrome (Raynaud's disease), which is usually mild, and secondary Raynaud's syndrome (Raynaud's phenomenon), which can be more severe.

Types

Both primary and secondary Raynaud's syndrome are characterized by the constriction of small blood vessels—usually on both sides of the body (bilateral)—that leads to decreased blood flow to the fingers, and sometimes toes, tip of the nose, earlobe, skin over the kneecap, or nipples.

Both primary and secondary Raynaud's syndrome are much more common in women than men, and they usually affect women during the childbearing years. Thought to affect roughly 5 percent of the population in the United States, Raynaud's more common in colder climates and in those who have a family history of the condition.

Primary Raynaud's Disease

Primary Raynaud's disease tends to occur in younger women than secondary Raynaud's, with a peak incidence between the ages of 15 and 25. It is differentiated from secondary Raynaud's in that there is no underlying medical condition, but a small percentage of people diagnosed with primary Raynaud's will later develop one of the conditions that underlie secondary disease (and the diagnosis will be changed).

The condition is usually mild, and can be controlled by lifestyle measures alone.

Secondary Raynaud's Phenomenon

Secondary Raynaud's syndrome tends to have a later onset than the primary condition, and it is commonly diagnosed around the age of 40. It is associated with a number of underlying causes, such as hypothyroidism, and can be more severe. In addition to lifestyle measures, medications and even surgical procedures may be needed to control the symptoms and prevent complications.

Signs and Symptoms

The signs and symptoms of Raynaud's syndrome can range from barely detectable to those that greatly limit activities and affect quality of life.

In terms of sensations, fingers (and for around 40 percent of people, toes), may become cold and numb as the blood flow is decreased with vasoconstriction (narrowing of the blood vessels).

Vasoconstriction: Step by Step

  1. The fingers often appear white (pallor).
  2. The fingers then take on a bluish appearance (cyanosis) as the tissues are deprived of oxygen and nutrients.
  3. As blood flow returns, the fingers or toes may become bright red (rubor).
  4. Symptoms of tingling, swelling, and then painful throbbing may occur (more common with secondary Raynaud's syndrome).

The symptoms may involve only one or more fingers, though it's uncommon for thumbs to be affected. "Vasospastic attacks" may affect different fingers or toes than previous episodes. Other areas of the body may be affected as well.

Triggers

Episodes of Raynaud's are not always predictable, but there are several common triggers including:

  • Going outside in cold temperatures
  • Putting hands in cold water
  • Putting hands in the freezer
  • Holding an iced drink
  • Emotional stress of any kind

Complications

For most people, Raynaud's syndrome is a nuisance and uncomfortable, but does not result in lasting damage. That said, and especially with secondary Raynaud's, complications may occur. These can range from skin ulcers that heal slowly to scarring, and—in very severe cases— gangrene that could lead to loss of digits.

Causes

Vasoconstriction in the extremities is a normal, protective response to cold exposure. It's your body's way of maintaining warmth in your core, where vital organs reside.

With primary Raynaud's syndrome, the constriction of blood vessels is an "extreme" normal reaction to cold. While the mechanism is not completely understood, it's thought that an "overactive" sympathetic nervous system is at play, and that these nerves are either oversensitive to cold or stimulate excessive contraction of blood vessels. Though there are no confirmed causes of primary Raynaud's, there are known risk factors (see below).

With secondary Raynaud's phenomenon, thickening of the blood vessel walls, or other changes, may magnify this effect. There are several conditions that are associated with secondary Raynaud's. With some of these, Raynaud's phenomenon is very common; for example, 85 percent to 90 percent of people who have scleroderma also experience Raynaud's. It is not as common with others.

Risk Factors

Risk factors for both types of Raynaud's syndrome include:

  • Female gender
  • Childbearing age
  • Cold climate
  • Family history of the condition
  • Smoking

Underlying conditions for secondary Raynaud's phenomenon include:

Connective Tissue/Autoimmune Diseases

Other Medical Conditions

Injuries/Trauma

  • Injuries or surgery to the hands or feet
  • A history of frostbite
  • Repetitive exposures on the job, especially with vibrating hand tools such as jackhammers
  • Other repetitive hand activities, such as playing the piano or typing on a keyboard
  • Carpal tunnel syndrome

Medications

  • Beta-blockers
  • Some chemotherapy drugs (cisplatin, vinblastine, bleomycin)
  • Some migraine medications (ergot derivatives and sumatriptan)
  • Some over-the-counter cold and allergy preparations
  • ADHD drugs
  • Estrogen-containing medications
  • Narcotics

Toxins

  • Vinyl chloride

Diagnosis

A diagnosis of Raynaud's syndrome begins with a careful history, including symptoms, triggers, and risk factors for the disease. While at first symptoms may suggest primary Raynaud's syndrome, the diagnostic process can sometimes discover underlying autoimmune conditions (there are more than 80 types) associated with secondary Raynaud's phenomenon.

For example, for roughly 30 percent of people with scleroderma, Raynaud's syndrome is the first symptom of the disease—and it may be the only symptom for many years.

Autoimmune Diseases and Types

Physical Exam

With primary Raynaud's, the physical exam is usually normal, unless an attack of Raynaud's is witnessed and the signs of vasoconstriction are seen. With secondary Raynaud's, examination may reveal evidence of a connective tissue disease or other condition.

Laboratory Tests

Laboratory tests are done primarily to look for underlying conditions. An antinuclear antibody test (ANA) test often done when autoimmune diseases or connective tissue disorders are suspected. With some of these diseases, people make antibodies against the nuclei of their own cells, which show up with a special stain.

An erythrocyte sedimentation rate (sed rate) or C-reactive protein test are non-specific tests that are a measure of inflammation in the body. Thyroid function tests may also be done.

Procedures

There are two procedures that may be done in the office to evaluate possible Raynaud's syndrome:

  • Nailfold capillaroscopy: This study involves placing a drop of oil at the base of a fingernail, and then examining the area under a microscope. With connective tissue diseases, the capillaries may be abnormal appearing. This test can, thus, be helpful in distinguishing between primary and secondary Raynaud's syndrome.
  • Cold stimulation test: In a cold stimulation test, heat sensors are taped to your fingers and your hands are then submerged into an ice-water bath. The device measures how quickly fingers warm up and return to normal temperature when removed from the water. With Raynaud's syndrome, the response is slow; it can take 20 minutes or more before fingers regain a normal temperature.

Diagnostic Criteria

A diagnosis of Raynaud's syndrome may seem obvious, but one cannot be formally made until certain criteria are met.

Diagnostic criteria for primary Raynaud's syndrome include:

  • Episodes of pallor (whiteness) or cyanosis (blueness) of fingers or toes that a) are triggered by exposure to cold or stress and b) have occurred for at least two years
  • Symmetric involvement of both sides of the body (for example, both hands)
  • The absence of complications such as skin ulcers, pitting scars, or gangrene of the fingers or toes
  • The absence of a known underlying cause
  • Normal lab tests for inflammation (negative ANA and normal sed rate)
  • Normal findings on nailfold capillaroscopy

Diagnostic criteria for secondary Raynaud's phenomenon include the presence of at least two of the following:

  • The same symptom attacks found with primary Raynaud's
  • An abnormal nailfold capillary pattern
  • A known underlying condition
  • Positive ANA
  • Positive sed rate
  • Scars, ulcers, or gangrene of the fingers or toes

Differential Diagnoses

There are a number of conditions that may appear similar to Raynaud's syndrome, but differ in the mechanism of the symptoms. These include:

  • Buerger disease (thromboangiitis obliterans): This condition is usually seen in men who smoke; pulses in the hand or feet may be decreased or absent. (When Raynaud's phenomenon occurs along with Buerger disease, it is usually in only one or two digits.)
  • Hyperviscosity (thick blood) syndromes, such as myeloproliferative diseases and Waldenstrom's macroglobulinemia.
  • Blood dyscrasias, such as cryoglobulinemia with multiple myeloma; cold can cause clumping of proteins, red blood cells, platelets, etc.
  • Atherosclerosis: Narrowing or occlusion of arteries in the extremities (peripheral vascular disease) may cause symptoms that are similar to Raynaud's, but persistent. Clots that break off and travel to the digits (emboli) can also cause occlusion of the blood vessels in the digits and tissue death.
  • Acrocyanosis: In this condition, the blueness of the digits is permanent rather than temporary.
  • Drug-related vasoconstriction, such as with some migraine medications, interferon, or cocaine.
  • Reflex sympathetic dystrophy

Treatment

The goals of treatment with Raynaud's syndrome are to reduce the frequency and severity of attacks and prevent complications. While lifestyle measures are often sufficient, medical treatment may be needed, especially with secondary Raynaud's.

Optimal treatment for the underlying condition in secondary Raynaud's syndrome is also critical. With conditions such as hypothyroidism, accurate and optimal treatment of the condition can significantly improve symptoms, as cold extremities are a symptom of undertreated thyroid disease as well. With connective tissue disorders, accurate treatment may reduce the damage to blood vessels that can lead to Raynaud's phenomenon.

Lifestyle Measures

Lifestyle measures are the main way to control the disease for the majority of people and involve avoiding the triggers that can result in an attack.

Protect Yourself in Cold Temperatures

Protect your hands with mittens or gloves, and your feet with thick socks (some people find it helpful to wear them outside as well as to bed). To prevent vasoconstriction of the extremities, be sure to keep your core warm as well. Wearing layers of clothing, scarves, and especially keeping your head covered (with a hat that covers your earlobes) to prevent heat loss is imperative.

Using chemical- or battery-operated hand or foot warmers can be helpful, as can keeping your hands warm by placing them in your armpits while wiggling your fingers. If you live in a colder climate, warming your car before leaving home can reduce your exposure, and it may pay to invest in a remote car starter.

Respond Promptly to an Attack

When an attack occurs, it's best to go indoors. Apply warm water by running your hands and/or feet (depending on the location of your symptoms) under the faucet, placing them in the bathtub, or soaking them in a bowl. Gently massage your hands or feet.

Avoid Certain Medications

A number of medications can cause vasoconstriction and should be avoided. This includes avoiding over-the-counter cold and allergy preparations that contain ingredients such as Sudafed (pseudoephedrine) and many more.

Some prescription drugs, such as oral contraceptives (the birth control pill), estrogen replacement therapy, and beta-blockers (for heart disease or high blood pressure), can worsen symptoms for some people.

Moderate Your Diet and Increase Exercise

Limiting alcohol and caffeine consumption is helpful. Moderate exercise can improve circulation and can be of benefit.

Practice Stress Management

Stress management is important for anyone, but much more so for those who have Raynaud's syndrome, as emotions can lead to attacks.

Explore Work Adjustments

If your job requires you to be outside, or if you must travel in cold weather to get to work, talk to your doctor. The Adults With Disabilities Act requires employers who have 50 or more employees to provide "reasonable accommodations" for those with some medical conditions. There are many ways in which this could help, such as checking out the option for working from home during particularly cold days.

Make Changes at Home

For people coping with Raynaud's, taking cold food items out of the refrigerator or freezer may precipitate an attack. You may wish to keep a pair of gloves in your kitchen for just this purpose, as well as in your car for when you go grocery shopping.

Keeping your eye on the thermostat is also key, as air conditioning is a common trigger for attacks. Cold beverages can also be a problem, and using insulated drinking glasses may help.

Quit Smoking

If you smoke, quit, as smoking causes vasoconstriction. Try to avoid secondhand smoke as well.

Protect Your Hands and Feet

Injuries to the hands or feet can increase your risk of complications from Raynaud's, as the healing of cuts, scrapes, and bruises can happen slowly. Wear shoes rather than going barefoot. If you develop dry hands or feet, especially foot cracks, use a good lubricating lotion or ointment to keep your skin moist. Also, avoid too-small rings, snug shoes, tight watches, and anything that constricts circulation to your hands and feet.

Medications

Medications may be used if lifestyle measures are inadequate, and they are most often needed for people with secondary Raynaud's syndrome. For some people, medications may only be needed during the cold winter months and can be stopped during the summer.

Calcium channel blockers, which work to dilate blood vessels, are some of the safest and most helpful medications used for Raynaud's. Of this category, Procardia (nifedipine), Norvasc (amlodipine), and Cardizem or Dilacor (diltiazem) appear to be most effective and can decrease both the frequency and severity of attacks; the slow release forms often work best. Calcium channel blockers may work best for primary Raynaud's disease, and sometimes higher doses are more effective.

In addition to calcium channel blockers, other categories of medications that have been effective for at least some people include:

  • Alpha-blockers, such as Minipress (prazosin), Cardura (doxazosin), and Hytrin (terazosin): These medications prevent the action of a hormone that constricts blood vessels.
  • Angiotensin-receptor blockers, such as Cozaar or Hyzaar (losartan), may decrease the severity of attacks.
  • Other sympatholytic drugs, such as Aldomet (methyldopa), Ismelin (guanethidine), and Dibenzyline (phenoxybenzamine)
  • Phosphodiesterase inhibitors can dilate veins and may ease symptoms. Examples include Viagra (sildenafil), Cialis (tadalafil), and Levitra (vardenafil).
  • Selective serotonin reuptake inhibitors, such as Prozac (fluoxetine)
  • Oral prostaglandins, such as Cytotec (misoprostol): For severe Raynaud's (such as if ulcers are present and amputation is feared), IV Flolan (epoprostenol) may be used.
  • The endothelin receptor inhibitor Tracleer (bosentan) paired with Ventavis (iloprost) may be helpful for severe Raynaud's related to scleroderma.
  • Mild blood thinners, such as aspirin or Persantine (dipyridamole), or drugs that help red blood cells flow better through the circulation, such as Trental (pentoxifylline)

    Topical nitroglycerin (ointment, paste, gel, or patches) may be helpful for those who hesitate to take a medication every day. A 2018 review in Rheumatology International found that most studies found nitroglycerine ointment to be beneficial. Nitroglycerine is a vasodilator and may be particularly useful when ulcers are present.

    As noted, there are many options, and one category of oral or topical treatment may work better for one person than another.

    If you have secondary Raynaud's, optimal treatment of the underlying condition (such as control of connective tissue disorders or optimal thyroid treatment) is essential, and medication may be a part of that.

    Procedures

    Procedures are used less frequently than medications, and are most often chosen when nothing else is working for symptoms or when complications such as ulcers occur. These treatments target the sympathetic nerves. Options include:

    • Sympathectomy: Digital sympathectomy is a surgical procedure in which the sympathetic nerves that cause the constriction of blood vessels are cut. It is used primarily for secondary Raynaud's syndrome. Cervical sympathectomy involves cutting these nerves higher up and is more effective for primary Raynaud's disease. The effect of these procedures may reduce symptoms, but the improvement is often transient.
    • Injections: Local injections of Botox, local anesthetics, or onabotulinumotoxin type A may interrupt the nerve signal that causes the vasoconstriction.

    Complementary and Alternative Medicine

    There are many mind/body therapies that are helpful in stress reduction, and therefore could reduce the frequency of your attacks. Some of these include:

    Biofeedback has helped some people to control attacks as well.

    A number of dietary and herbal supplements have been evaluated with regard to Raynaud's syndrome, but the science is still young. There is some early evidence that fatty acid supplements could be helpful.

    Niacin (vitamin B3) dilates blood vessels and may help, but use is often limited by side effects such as diarrhea and flushing. Gingko biloba has also been looked at but was not as helpful as nifedipine in one study. There are also some researchers who believe oral L-arginine may be of benefit with secondary Raynaud's phenomenon.

    Prognosis

    The prognosis of Raynaud's syndrome depends on whether it primary or secondary, and the underlying processes when the latter. Most people with mild disease do very well and complications are uncommon.

    For roughly 15 percent of people, the disease spontaneously goes away in time, while around 30 percent will have symptoms progress (perhaps involving all fingers instead of a fingertip). For the remainder of people, there is no clear pattern of change or progression of the disease.

    Coping

    Coping with Raynaud's syndrome can be frustrating, as it can cause people to significantly limit activities to avoid attacks. With secondary Raynaud's phenomenon, the underlying disease can also be challenging. Taking an active role in your care can help you feel more in charge of your disease. Take time to learn about your disease. Lean on your family and friends for support.

    With conditions such as Raynaud's syndrome, it can be very helpful to connect with others who are also coping with the disease. Organizations such as the Raynaud's Association provide support and education for many people with the condition. There are several Facebook groups available as well. For those who are active on social media, the hashtag #Raynauds can help you find others who have similar questions and learn about what has best helped others cope.

    How Social Support Contributes to Psychological Health

    A Word From Verywell

    An accurate diagnosis of Raynaud's syndrome is important both in controlling the symptoms and preventing complications. Evaluation of what's believed at first to be primary Raynaud's disease may also lead to the diagnosis of an underlying condition that can then be treated.

    If you've been diagnosed, putting attention toward the details of your symptoms and their triggers is invaluable. These clues can help you know which lifestyle adjustments may help you most.

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