Everyone knows what happens when we forget our gloves in freezing cold weather. Our fingers quickly get cold, white, and numb. This is normal—the body closes off the blood flow to the extremities to conserve heat. Suppose, however, it happens when reaching into a freezer or walking into an air-conditioned room. Welcome to the world of Raynaud’s disease, an overactive cold response that is perhaps the most common medical condition no-one has ever heard about.
Raynaud’s disease (or Raynaud’s phenomenon) is an exaggerated cold response. The body shuts down blood flow to the fingers, toes, or sometimes ears, nose, or nipples when the body is exposed to cold or stress that would not normally provoke a cold response.
Raynaud’s is very common, but not exactly a household name. About one in twenty Americans have the condition, but the incidence may be as high as one in five among women. For many, the condition is little more than a nuisance. For others, however, it can substantially interfere with daily life and even represent a health threat.
Raynaud’s is not exactly a “disease,” but rather it is a symptom of other conditions that may be more serious. Most commonly, Raynaud’s is the first manifestation of systemic scleroderma, a connective tissue disease similar to rheumatism. But Raynaud’s can be caused by a wide variety of various conditions and diseases. Raynaud’s, then, is less a diagnosis than a mystery to be solved.
To understand Raynaud’s, it is best to start with understanding the body’s cold response. The body protects itself from cold temperatures through a complex cascade of reactions. When skin nerves register cold temperatures, the sympathetic nervous system (the part of the nervous system in charge of involuntary muscles) provokes the release of substances that cause arteries to narrow. The goal is to block blood flow to conserve heat.
The small arteries, called arterioles, in the fingers and toes are narrow to begin with, so there’s not much room left when they constrict. Blood flow through the arterioles becomes blocked (ischemia), causing the fingers or toes to get white, numb, and feel like they’re being stuck with pins and needles. Over time, the skin tissues become deoxygenated and turn blue. When the fingers are warmed up, the blood flows back, turning the fingers bright red.
These effects are also the symptoms of Raynaud’s, except, rather than being provoked by extreme cold, this cold response can be triggered by an air-conditioned room or picking up an iced tea. Cold isn’t even necessary. One-third of Raynaud’s attacks are triggered by emotional stress, another condition that causes blood vessel constriction.
Raynaud’s attacks typically hold on longer than they do with normal cold responses. The white or blue phase of a Raynaud’s attack typically lasts for 20 minutes. In more severe cases, attacks can last for hours after the triggering event.
What causes Raynaud’s? For most people, the cause is “functional vasospastic disorder.” Called primary Raynaud’s, this vasospasm condition is mild, often temporary, and not a health threat. Primary Raynaud’s usually begins between the ages of 15 to 25, primarily affects women, and is treated with lifestyle changes to keep the body warm and avoid triggers.
Secondary Raynaud’s is a manifestation of other medical conditions, and this is where the story gets more complex. Secondary Raynaud’s is typically more serious. Attacks are more frequent, more severe, longer-lasting, and could cause sores, scars, and other tissue damage such as gangrene. For all these reasons, a physician needs to track down the underlying cause. These are the possibilities a healthcare provider has to sort through when diagnosing Raynaud’s:
The first visit to the general practitioner or internist will involve a history and a physical exam. Before seeing a healthcare provider, patients should be ready to answer the following questions:
The healthcare provider may examine the hand for any evidence of blood vessel damage. They might also place the hand in cold water to observe the color changes, symmetry, and progression of a Raynaud’s attack.
The healthcare professional may also assess for risk factors, not just for Raynaud’s, but to search out a possible cause. People most at risk for primary Raynaud’s are female, under the age of 30, and live in colder climates. People most at risk for secondary Raynaud’s have evidence of a connective tissue disease, smoke, perform repetitive actions such as typing, or take certain medications.
If there is enough evidence for Raynaud’s, a referral to a rheumatologist will follow. Doctors of rheumatology specialize in autoimmune diseases such as scleroderma. The rheumatologist will perform blood tests, tests for antibodies typical of autoimmune diseases (an ANA or antinuclear antibody test), and will examine the finger’s capillaries using nailfold capillaroscopy. This microscopic examination of the capillaries along the fingernails will show any capillary damage typical of scleroderma or other connective tissue diseases.
A primary care physician can treat primary Raynaud, but secondary Raynaud’s will typically be treated by a rheumatologist. Neither primary nor secondary Raynaud’s can be cured, however, so treatment will be focused on limiting the frequency and severity of attacks. Most patients with primary Raynaud’s can be treated with lifestyle changes only, but more severe cases may require medications or even surgery.
There is no blood test or X-ray for Raynaud’s, so healthcare providers can only determine the effectiveness of Raynaud’s treatment based on the patient reports. Many doctors will have patients use a Raynaud Condition Score (RCS) diary that measures the frequency and severity of attacks, quality of life, and how the condition affects the patient day-to-day.
The first step in Raynaud’s treatment is to remove any possible cause for the condition, such as the use of vibrating machinery, smoking, medications, or chemical exposure at work.
Whether diagnosed with primary or secondary Raynaud’s, patients will be asked to make lifestyle changes to better manage the condition. These include avoiding cold, wearing warmer clothing, exercising, and managing stress.
Raynaud’s is a condition in which arterioles in the extremities constrict. Vasodilators, or medications that widen blood vessels, may reduce the number and severity of attacks.
In the most severe cases that don’t respond to medications, surgery may be required to rescue the fingers. A sympathectomy removes the sympathetic nerves that control vasoconstriction in the fingers and reduces symptoms in nine in ten patients.
Raynaud’s, a condition due to vasoconstriction, is treated by vasodilators. Calcium channel blockers are the first-line therapy, but more severe cases may require more powerful or more direct vasodilators such as PDE (phosphodiesterase) inhibitors, nitroglycerin, or, in very rare cases, prostaglandins.
Calcium channel blockers (CCBs) are the first-line medication for the treatment of Raynaud’s disease. These medications, such as amlodipine and nifedipine, work by relaxing blood vessel muscles. Your doctor may try other blood pressure medications such as ACE inhibitors, angiotensin receptor inhibitors (losartan), or alpha-blockers (prazosin). For patients with systemic sclerosis and severe Raynaud’s, bosentan, a drug prescribed for pulmonary high blood pressure, may be used.
Best-known under brand names such as Viagra or Cialis, PDE inhibitors dramatically increase the width of arteries and arterioles and increase blood flow. They work by preventing the breakdown of nitric oxide, a small molecule that causes the smooth muscles in arteries to relax. PDE inhibitors such as sildenafil or vardenafil can be prescribed along with calcium channel blockers or used alone.
Nitrates, such as nitroglycerin, are “direct vasodilators” that deliver nitric oxide directly to the arteries and veins, causing them to widen and blood flow to increase. The most common is nitroglycerin gel applied directly to the affected areas on the skin.
Prostaglandins are naturally produced by the body in response to an infection or allergen and some are powerful vasodilators. In very rare and severe cases of Raynaud’s caused by systemic scleroderma, prostaglandins, such as iloprost, are used off-label and injected intravenously. A prostaglandin injection can provide higher cold tolerance and fewer attacks for a few weeks.
Most patients with primary Raynaud’s can manage the condition without medications. Patients with secondary Raynaud’s, however, may need medications to reduce attacks. The first-line and usually the most effective medication for Raynaud’s are calcium channel blockers, a vasodilator used to treat hypertension (high blood pressure) and angina (chest pain).
Best medication for Raynaud's disease | ||||
---|---|---|---|---|
Drug Name | Drug Class | Administration Route | Standard Dosage | Common Side Effects |
Procardia (nifedipine) | Calcium channel blocker | Oral | One 10 mg capsule three times per day | Dizziness, flushing, headache |
Norvasc (amlodipine) | Calcium channel blocker | Oral | One 10 mg tablet taken once per day | Swelling, headache, fatigue |
Viagra (sildenafil) | PDE (phosphodiesterase) inhibitor | Oral | One 100 mg tablet per day in divided doses | Headache, flushing, stomach upset |
Nitro-Bid (nitroglycerin cream) | Vasodilator | Topical | ½ inch (7.5 mg) of 2% ointment applied to the most severely affected fingers for 6 to 12 hours | Headache, flushing, lowered blood pressure |
Many of the standard dosages above are from the U.S. Food and Drug Administration (FDA) and the National Institutes of Health (NIH). Dosage is determined by your doctor based on your medical condition, response to treatment, age, and weight. Other possible side effects exist. This is not a complete list.
Raynaud’s is primarily treated with vasodilators, or medications that cause blood vessels to relax and widen. Vasodilators are typically used to treat high blood pressure, so the most common side effect of all Raynaud’s medications is to lower blood pressure. The most serious side effect, of course, is a dangerous and life-threatening drop in blood pressure called hypotensive shock. For this reason, patients who already have low blood pressure will not be able to use these drugs. Other typical side effects of vasodilators include flushing, headache, edema, and heart palpitations.
In addition to lowering blood pressure, calcium channel blockers slow down the heart. As a result, they can also cause weakness, dizziness, and fatigue.
PDE inhibitors cause many of the same side effects as blood pressure medications. Headaches are very common. PDE inhibitors are most commonly used to treat erectile dysfunction, so painful or long-lasting erections can also be a side effect.
The most common side effect of nitrates is headache, often quite painful. In fact, most patients who use nitrates will experience a headache. Other side effects include nausea and dizziness. Topical nitrates rapidly lose their effectiveness, also, so they can only be used for 12 hours each day.
Most cases of Raynaud’s will involve lifestyle changes that involve avoiding triggers and keeping warm.
When it’s cold outside, bundle up. Coat, hat, scarf, winter socks, winter underwear, mittens, and warm boots; the more the better.
Keeping the entire body warm is a proven way to reduce the frequency and severity of Raynaud’s attacks. Wear warm clothing indoors. In the summertime, be ready to put on clothing before entering an air-conditioned building; the cold shock might trigger an attack. Socks should be on at all times. Wear mittens or gloves when removing items from the refrigerator or freezer.
Air conditioning may feel good, but a colder environment means more attacks, so it may need to be dialed up to a warmer temperature. During the winter, make sure the car interior is warm before you drive off.
Use insulated glasses for cold drinks. It not only helps prevent Raynaud’s attacks but also has the added benefit of keeping the drink cold longer.
Stress causes one in three Raynaud’s attacks. Avoiding stress is not always possible, but stress can be reduced through relaxation activities, meditation, exercise, good sleep hygiene, and avoiding stimulants.
Physical activity improves blood flow and warms the body.
Smoking can cause Raynaud’s. Quitting not only reduces Raynaud’s attacks, but it also improves overall physical and mental health.
For most people, Raynaud’s is a mild condition. In some, however, it may be a symptom of a more serious disease, such as systemic scleroderma. In the most severe but very rare cases, Raynaud’s can cause ulcers and even gangrene on the fingers.
During a Raynaud’s attack, circulation can be improved by warming up. If you are in the cold, move to a heated area to warm up the body. Warm up the affected area by placing it under warm water, under the arms, or rubbing them together.
For many people, Raynaud’s is a lifelong condition. However, in nearly half the patients with primary Raynaud’s, the condition does spontaneously remit over time.
Certain medications that cause blood vessels to constrict can also cause Raynaud’s, such as:
Raynaud’s is not a disease, but a symptom of an underlying condition. By itself, primary or secondary Raynaud’s does not affect the heart or any other organ. There are underlying conditions that cause Raynaud’s, such as systemic scleroderma, lupus, or cryoglobulinemia, that can affect the heart or other organs.
Raynaud’s is often the first clinical manifestation of certain connective tissue autoimmune diseases such as systemic scleroderma, lupus, Sjogren’s syndrome, or rheumatoid arthritis.
Raynaud’s attacks are triggered by cold and stress. Approximately one in three Raynaud’s attacks are due to stress or surprise.
Raynaud’s is a vasoconstrictive condition, that is, it causes blood vessels to contract. Medications that treat Raynaud’s are vasodilators—they cause blood vessels to widen. The first-line medications for moderate to severe cases of Raynaud’s are calcium channel blockers such as nifedipine and amlodipine. Normally used to treat high blood pressure, calcium channel blockers relax blood vessels to increase blood flow.
Patients with Raynaud’s should avoid medications that constrict blood vessels, including migraine medications (ergotamine and triptans), over-the-counter cold and allergy medications, diet aids, beta-blockers, and birth control pills. It may not be possible to completely boycott these medications, but taking them raises the risk for Raynaud’s attacks.
The goal of any therapy for Raynaud’s is to keep the blood vessels open. Medications that treat Raynaud’s are all vasodilators, which dilate, or widen, the blood vessels. The molecule responsible for vasodilation is nitric oxide, so vitamins and nutrients that boost nitric oxide in the body will help reduce the frequency and severity of Raynaud’s. These include antioxidants (vitamin C or vitamin E), L-arginine, and nitrate-rich vegetables.
Dr. Anis Rehman is an American Board of Internal Medicine (ABIM) certified physician in Internal Medicine as well as Endocrinology, Diabetes, and Metabolism who practices in Illinois. He completed his residency at Cleveland Clinic Akron General and fellowship training at University of Cincinnati in Ohio. Dr. Rehman has several dozen research publications in reputable journals and conferences. He also enjoys traveling and landscape photography. Dr. Rehman frequently writes medical blogs for District Endocrine (districtendocrine.com) and hosts an endocrine YouTube channel, District Endocrine.
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