Diseases

Raynaud’s Phenomenon

Raynaud’s Phenomenon (Raynaud’s, or RP) is a condition where very small blood vessels go into spasm or “clamp down” in response to cold temperatures.

Raynaud’s reduces blood flow to the extremities, usually the fingers and toes, causing them to turn white and feel cold. When they warm back up, the skin usually turns another colour such as blue or red before returning to normal.

Causes of Attacks

Attacks of Raynaud’s are usually caused by exposure to cold temperatures and can last anywhere from a few minutes to a few hours or longer. Other things that can trigger attacks include emotional stress, trauma (injury), hormonal changes, and smoking.

People have a higher risk of developing Raynaud’s if they have had a previous injury to the extremities like frostbite or surgery, as well as those with a history of repetitive actions or vibrations, such as using a jackhammer, drills, typing, or playing the piano.

Primary and Secondary Types

There are two basic types of Raynaud’s: primary and secondary.

Primary Raynaud’s

In primary Raynaud’s there is no associated underlying autoimmune disease. This type of Raynaud’s is seen on its own and happens by itself. It usually affects young women in their 20s or 30s and appears to be more common in young women who are thin. The disease often runs in families.

Secondary Raynaud’s

Secondary Raynaud’s is “secondary” to an autoimmune disease like systemic sclerosis (scleroderma), lupus, rheumatoid arthritis, and Sjogren’s syndrome. Secondary Raynaud’s tends to be more severe than primary Raynaud’s, start at an older age, and the symptoms may get worse over time.

Secondary Raynaud’s is associated with underlying autoimmune diseases to varying degrees. It has been estimated that almost all people with systemic sclerosis (scleroderma) also have Raynaud’s, and that 1 in 3 people with Sjogren’s syndrome have it.

It is not understood what causes the blood vessels to become more sensitive to cold temperatures and other triggers. Some experts think the answer might be related to the immune system because Raynaud’s can be associated with autoimmune or connective tissue diseases like as rheumatoid arthritis and scleroderma.

Effect on Pregnancy

Pregnancy can be more complicated for women with Raynaud’s and an underlying autoimmune disease. Depending on the specific disease or syndrome, these women may be at risk of multiple miscarriages.

Raynaud’s can rarely affect a woman’s ability to breastfeed in cases where the nipple area is affected.

Understanding Raynaud’s Phenomenon

Symptoms

Raynaud’s Phenomenon is often triggered by cold temperatures or in stressful situations. People with Raynaud’s can experience an “attack” of symptoms where blood flow to the fingers and toes is significantly reduced. Some people will experience colour changes in affected areas, which can turn white (pallor), blue (cyanosis), and/or red (rubor) over the course of an attack.

The areas where blood flow is reduced can be quite painful and a burning feeling may be present as the areas re-warm.

Most attacks last around 5 to 10 minutes but they can vary in duration. Some may be very brief, lasting only a minute or two, while others may be much longer, lasting up to several hours.

It is possible to have more than one attack per day.

The areas that are most commonly affected by Raynaud’s are the fingers and the toes. Less commonly, it can also affect the nose, ears, lips and nipples.

In severe cases of secondary Raynaud’s, when the condition is accompanied by an autoimmune or connective tissue disease, it can cause painful ulcers on the fingertips.

Diagnosis

Raynaud’s phenomenon is best diagnosed by a rheumatologist, a type of doctor that specializes in arthritis and autoimmune disease.

To diagnose Raynaud’s, they will take a careful and complete history focusing on the nature of their patient’s Raynaud’s attacks, and perform a thorough physical examination. Based on this information, the doctor will likely order tests to confirm the diagnosis, investigate if the Raynaud’s is primary (it appears on its own) or secondary to an undiscovered autoimmune disorder, and to rule out other possible conditions.

The most pertinent sign of Raynaud’s is the lack of blood flow to the fingers or the toes during an attack. This can be seen easily as the area affected will turn white, the red or blue then return to the natural skin colour.

The best way to diagnose Raynaud’s is to ask the patient to bring in pictures of the affected area to observe colour changes.

Another method to test for Raynaud’s is known as the “cold challenge”. A person simply places their hands under cold water. Those with Raynaud’s will lose circulation to their hands and fingers much more quickly than those not affected. A doctor observing the challenge can tell the difference between normal circulation and the reduced circulation seen with Raynaud’s. This is not done frequently as it is uncomfortable for the patient.

Common Tests to Diagnose Raynaud’s Phenomenon

The following tests are commonly used to help diagnose Raynaud’s. Other more specific tests might be performed to investigate if there are potential underlying autoimmune diseases that might accompany secondary Raynaud’s.

“Cold Challenge” Test

The “cold challenge” test involves the patient placing their hands under cold water, with a doctor observing for signs of reduced circulation.

Blood Tests

Looking for inflammation: These tests may produce abnormal results in patients with secondary Raynaud’s, where the condition is accompanied by an autoimmune or connective tissue disease that causes inflammation in the body. Common tests include a Complete Blood Count (CBC), Erythrocyte Sedimentation Rate (ESR), and C-Reactive Protein (CRP).

Looking for signs of autoimmune disease: Rheumatoid Factor (RF), Anti-Nuclear-Antibody (ANA), and other tests that look for signs of autoimmune diseases can help identify secondary Raynaud’s.

To rule out thyroid conditions: the Thyroid Stimulating Hormone (TSH) test rules out thyroid conditions that might be associated with a patient’s symptoms.

Scans

Thermography (thermal imaging) is used in research settings to understand how a patient’s circulation is affected in their extremities. In most patients, the “cold challenge” with cold running water is sufficient to diagnose Raynaud’s.

Causes

Scientists don’t fully understand what causes blood vessels to become more sensitive to cold temperatures and other triggers, such that Raynaud’s phenomenon is developed.

Some experts theorize that Raynaud’s phenomenon might be related to the immune system because it is sometimes associated with autoimmune or connective tissue diseases such as rheumatoid arthritis, scleroderma, Sjogren’s syndrome and lupus.

Attacks of Raynaud’s are usually caused by exposure to cold temperatures. Many other things can trigger an attack too, including emotional stress, trauma (injury), hormonal changes, and smoking.

People have a higher risk of developing Raynaud’s if they have had a previous injury to the extremities like frostbite or surgery, as well as those with a history of repetitive actions or vibrations, such as using a jackhammer, drills, typing, or playing the piano.

Treatments

The most common treatment for Raynaud’s is making accommodations to avoid attacks, including keeping the body, hands, and feet warm. This can also involve avoiding emotional and environmental stress, vibrations, repetitive motions and activities, and lifestyle changes. Medications are only used when these measures do not provide sufficient control of symptoms.

Accommodations to Avoid Attacks

Keeping Warm

One of the best things a person with Raynaud’s can do to manage their symptoms is to keep warm. It is important to keep the whole body warm, not just the hands and feet. When the whole body is warm, it’s easier for the heart to pump warm blood to the extremities.

A warm hat (toque), scarf, and gloves are recommended in colder months. Wearing long underwear, sweaters, scarves, mittens or gloves, and socks can help any time depending on the climate.

It can be helpful to always wear gloves or mittens when handling cold objects, such as taking something out of the freezer.

Making Accommodations in Life and Work

Making certain accommodations help avoid attacks, such as wearing protective gloves in a cold work environment, or avoiding the use of vibrational tools like drills or jackhammers. It is advisable to reduce or avoid jobs or activities that involve repetitive motions.

It is also important to avoid stress and learn to cope with emotionally stressful situations.

Lifestyle and Health Changes (Caffeine, Smoking, Certain Medications)

Caffeine, nicotine, and other stimulants can trigger attacks or make Raynaud’s symptoms worse. It is wise to quit or at least significantly reduce the intake of these substances.

Smoking in general can trigger vasospasm (a sudden constriction of blood vessels) in the arterioles (a small blood vessel), which can exacerbate (intensify) the condition.

Sympathomimetic drugs should be avoided unless supervised by a doctor. These are drugs that affect the sympathetic nervous system. They include illegal drugs like cocaine and methamphetamine, certain supplements like Ephedra, cold medications that contain pseudoephedrine or oxymetazoline, and stimulant treatments for Attention Deficit Hyperactivity Disorder and narcolepsy.

Medications that Treat Raynaud’s Phenomenon

Most people with Raynaud’s who need medication only take it when they need it, or as instructed by their doctor. In colder climates, a lot of patients who need medication find that they only need to take it regularly during the winter when they know they will be spending extended amounts of time exposed to colder temperatures.

Calcium Channel Blockers

The first choice of medication to treat Raynaud’s is a type of antihypertensive (blood pressure lowering) medication called a calcium channel blocker. Examples include long-acting medications such as nifedipine, amlodipine, or felodipine.

Other Medications for Raynaud’s

If a calcium channel blocker isn’t sufficient to control symptoms or if a person can’t tolerate them, there are many other options to try.

Medications known for erectile dysfunction such as Viagra (sildenafil) or Cialis (tadalafil) can be effective in lower doses.

Losartan is another type of blood pressure medication that has been useful in some people with Raynaud’s.

Selective serotonin reuptake inhibitors (SSRIs) such as fluoxetine, a type of medication best known for as a treatment for depression; and alpha blockers such as prazosin, have also been used to improve RP symptoms.

In very severe cases of RP that have caused ulcers or gangrene (a serious condition where cells die due to lack of blood flow), a medication called iloprost may be given by intravenous infusion in a hospital setting to dilate (open) the blood vessels.