A person with Raynaud's disease experiences pain in the extremities, for example, the fingers, when temperatures drop.
Blood vessels narrow and almost completely shut down. Fingers or toes turn from white to blue and, then, as the blood returns, they flush red.
Also known as Raynaud's syndrome or Raynaud's phenomenon, Raynaud's disease affects 5 to 10 percent of Americans, but only 1 in 10 seek treatment. Females are an estimated nine times more likely to be affected than males.
Fast facts on Raynaud's disease
Here are some key points about Raynaud's disease. More detail and supporting information is in the main article.
- Raynaud's disease is caused by peripheral blood vessels overreacting to cold.
- The condition affects 5-10 percent of Americans.
- Maurice Raynaud first described the disease in 1862.
- Females and people living in colder climates are more often affected.
- A capillaroscopy can help diagnose Raynaud's disease.
There is no cure for Raynaud's disease, but there are ways to manage symptoms.
For mild forms of Raynaud's disease, covering exposed skin before leaving the house can help. If an attack occurs, soaking the affected parts in warm, not hot, water can alleviate symptoms and prevent them from worsening.
If stress is a factor, learning to manage stress can help.
For moderate to severe cases, medication may be necessary.
Alpha-1 blockers can counter the effect of norepinephrine, which constricts blood vessels. Examples include doxazosin and prazosin.
Dihydropyridine calcium channel blockers relax the smaller blood vessels of the hands and feet. Examples include amlodipine, nifedipine, and felodipine.
Other vasodilators dilate the veins, easing symptoms. Examples include losartan, sildenafil (Viagra), fluoxetine (Prozac), and prostaglandin.
In very severe cases, more invasive procedures are an option:
Nerve surgery: Sympathectomy
The vasoconstriction that causes Raynaud's is controlled by sympathetic nerves in the affected areas. A surgeon can make small incisions and strip the nerves away from the blood vessels, to decrease the frequency or severity of attacks. This is not always successful.
Injecting certain chemicals that block sympathetic nerve fibers from carrying out vasoconstriction can be effective. Local anesthetics or onabotulinumtoxin type A, or Botox, work well for some people. However, the effect will wear off, and treatment will need repeating.
Living with Raynaud's
People who are prone to Raynaud's can take measures to avoid some triggers.
The National Heart, Lung, and Blood Institute (NHLBI) suggest:
- wrapping up and keeping the house warm when temperatures are cold
- as far as possible, avoiding emotional stress
- exercise to promote a healthy lifestyle and reduce stress
- avoiding medicines and substances that trigger the symptoms
- limiting consumption of caffeine and alcohol
- not smoking
They also suggest following up with a physician, especially if sores develop on the extremities. Getting medical help may prevent a worsening of symptoms and serious complications.
Exactly what causes Raynaud's remains unclear, but a hyperactivation of the sympathetic nervous system is known to cause an extreme narrowing of the blood vessels, known as vasoconstriction.
It can happen when the person enters a cold place, opens a freezer, or puts their hands in cold water.
Some people experience symptoms when faced with stress, even without an associated drop in temperature.
In healthy individuals, the circulatory system in the extremities, such as the fingers and toes, reacts to conserve heat in cold conditions. The small arteries that supply the skin with oxygen narrow to minimize the amount of heat lost through the exposed skin surface.
In people with Raynaud's disease, this narrowing is excessive. This is what causes the blood vessels almost to shut down.
There are two types of Raynaud's disease: Primary and secondary.
Primary Raynaud's, or Raynaud's disease, is more common, and it affects people who do not have a secondary medical condition.
Secondary Raynaud's, or Raynaud's phenomenon or syndrome, results from an underlying medical issue. It is less common and tends to be more serious.
Causes of secondary Raynaud's
Causes of secondary Raynaud's include:
Diseases of the arteries: Atherosclerosis, a build-up of plaque in blood vessels, or Buerger's syndrome, a disorder where blood vessels in the hands and feet become inflamed, can cause Raynaud's symptoms. Primary pulmonary hypertension has also been linked to the disease.
Diseases of the connective tissue: Most people with scleroderma, a disease leading to hardened skin, have Raynaud's disease. The symptoms are commonly linked to lupus, rheumatoid arthritis, and Sjogren's syndrome, an autoimmune disease affecting glands.
Repetition or vibration: People whose hobbies or jobs require repetitive movements, such as typing or playing the guitar or piano, are at risk of developing Raynaud's symptoms. Those whose jobs involve using vibrating tools, such as a jackhammer, are also at risk.
Carpal tunnel syndrome: This condition puts pressure on the nerves traveling to the hand, and it increases susceptibility to Raynaud's symptoms.
Medications: Drugs that can induce Raynaud's include beta blockers, migraine medications containing ergotamine or sumatriptan, ADHD medications, some chemotherapy drugs, and some over-the-counter cold remedies.
Exposure to certain substances: Smoking narrows blood vessels and is a possible cause of Raynaud's. Other chemicals, such as vinyl chloride, may also play a role.
Injuries: Raynaud's can start after injuries such as frostbite, a broken wrist, or local surgery.
Raynaud's tends to affect females more than males. Primary Raynaud's normally starts between the ages of 15 years and 25 years, and secondary Raynaud's between 35 and 40 years.
The condition appears to run in families. A person a first-degree relative with Raynaud's is more likely to develop it.
Possible pathways of Raynaud's disease
The exact pathways of Raynaud's disease are not known, but it probably results from a combination of factors, and the combination of factors probably differs between individuals.
Nitric oxide is a strong vasodilator. It has been found in people with secondary Raynaud's but less so in those with primary Raynaud's disease.
Endothelin-1 is a potent vasoconstrictor. People with primary Raynaud's have been found to have high levels of this protein.
Serotonin, a neurotransmitter, has been found in high concentrations in people with Raynaud's
Platelet activation appears to be higher in people with Raynaud's. Platelets are components in blood that clump together to help prevent bleeding.
Fibrinolysis is a natural reaction that takes place in the body to reduce clotting. Some studies have linked low levels of fibrinolysis with Raynaud's.
Oxidative stress is caused by an increase in free radicals, and it could be involved in Raynaud's.
Raynaud's disease affects some people when they are exposed to the cold.
When temperatures drop, the blood vessels contract in the fingers or toes. This contraction causes hypoxia, or lack of oxygen, to the affected tissues. Fingers and toes will feel cold to the touch and possibly numb.
Often, the affected area will turn white, then later turn blue. Once the area is warmed and blood flow returns, a tingling sensation may accompany a red flush and, possibly, swelling. There may also be a painful, throbbing sensation.
Toes and fingers are most commonly affected, but Raynaud's can affect the nose, lips, and ears.
Some women may experience Raynaud's phenomenon of the nipples, especially during breastfeeding. It causes severe throbbing, similar to that experienced with a fungal Candida albicans (C. albicans) infection, which can lead to misdiagnosis.
An episode normally lasts around 15 minutes, including the time it takes for the body to normalize.
Raynaud's syndrome can affect either the hands or feet, or both.
To reduce the risk of an attack, it can help to keep the feet and hands warm, avoid smoking, and get enough exercise.
If an attack starts, it may be reduced or prevented by warming the hands and feet at once, for example, by massaging them.
The feet and hands should be protected from cuts, bruises, and other injuries as far as possible, because the lack of circulation can make it harder for them to heal. Use lotion to prevent the skin from cracking, and make sure you have comfortable, well-fitting shoes.
The physician will ask the patient questions about symptoms, diet, habits, and hobbies. The patient should be ready to explain when the symptoms first appeared, how often they have them, and what seems to cause them. It may help to keep a diary of this information.
To differentiate between secondary and primary Raynaud's, the doctor may do a capillaroscopy. This involves viewing the skin at the base of the fingernail and inspecting it under a microscope to assess for malformed or enlarged capillaries.
A blood test may be done to look for antinuclear antibodies or to measure erythrocyte sedimentation rate, which may indicate autoimmune problems or other inflammatory process.
Raynaud's is not normally life-threatening, but complications can occur.
Chilblains happen when there is a problem with the blood circulation, and Raynaud's is one possible cause. The skin becomes itchy, red, and swollen and it may feel hot, burning, and tender. Chilblains usually resolve in 1 to 2 weeks, but they can come back. Keeping the extremities warm can help prevent them. If the hands and feet become cold, warm them slowly, as too much heat can cause further damage.
If symptoms worsen and blood supply is substantially reduced for a long time, fingers and toes can become deformed.
If oxygen is completely cut off from the area, skin ulcers and gangrenous tissue can develop. Both of these complications are difficult to treat. They may eventually require amputation.