What Is Raynaud's Disease? What Causes Raynaud's Phenomenon?

Editor's Choice
Main Category: Vascular
Also Included In: Dermatology;  Pain / Anesthetics
Article Date: 21 Jan 2010 - 0:00 PDT

email to a friend   printer friendly   opinions  

Raynaud's disease, also known as Raynaud's phenomenon and sometimes simply Raynaud's, is a condition that causes some areas of the body to feel numb and cool in response to cold temperatures or emotional stress, caused by a problem with the blood supply to the skin. Raynaud's disease is a vasospastic disorder - spasms in the blood vessels lead to vasoconstriction (narrowing).

The disease mostly affects the fingers, toes, tip of the nose and the ears. The problem is in the blood vessels that supply the skin. Smaller arteries narrow and limit blood circulation to affected areas. Areas of the body subsequently become cold and very pale. Patients typically feel pins and needles, numbness, and even burning. The sensation can be unpleasant and painful.

According to Medilexicon's medical dictionary:

Raynaud's phenomenon is an idiopathic paroxysmal bilateral cyanosis of the digits due to arterial and arteriolar contraction; caused by cold or emotion.

The disease is named for Maurice Raynaud (1834 - 1881), a French physician who first described it in 1862.

The condition is either:

• Primary Raynaud's. This is the most common form, there is no apparent cause (idiopathic). It is possible for the primary form to move to the secondary form.
• Secondary Rayndaud's. It is associated with an underlying disease, such as rheumatoid arthritis. In extreme cases this form can progress to necrosis or gangrene of the fingertips.

Doctors may measure hand-temperature to distinguish between primary and secondary forms of the condition.

The National Health Service (NHS), UK, says that there could be as many as 10 million British people affected by Raynaud's disease. The NHS adds that a significant number of individuals never see their doctor about it because they think it is just part of their makeup, not knowing it is a disease with a name and treatment.

Raynaud's disease will usually affect people during or after middle age, although it can develop in all age groups. The majority of sufferers are women. As cold temperatures is one of the possible triggers, the condition becomes more common the further you move from the equator.

Most people have mild symptoms and do not find their daily tasks or general quality of life is affected. Even if symptoms are more severe, treatment (with medications) is usually effective.

What are the signs and symptoms of Raynaud´s disease?

A symptom is something the patient feels and reports, while a sign is something other people, such as the doctor detect. For example, pain may be a symptom while a rash may be a sign.

Generally, primary Reynaud's disease symptoms are mild, while the secondary form's may be more severe. In the primary form both hands are affected simultaneously, usually all fingers at the same time. In the secondary form the development and appearance of symptoms is more patchy, with perhaps a couple of fingers on one side being affected.

The affected areas will become very pale (pallor), and then take on a bluish color (cyanosis) due to hypoxia (lack of oxygen to that area). They will feel very cold and numb. If all fingers are affected, trying to rummage in your pockets for specific coins becomes much more difficult. This can sometimes be distressing.

When the episode subsides and bloodflow returns to the affected area the skin may turn red (rubor). During the recovery period there may be tingling and swelling.

Meanings in medicine:

• Pallor - whiteness.
• Cyanosis - a bluish color of skin (or mucous membranes) due to lack of oxygen in the blood.
• Rubor - redness

Not all sufferers go through the three color process - pallor, cyanosis, rubor - especially those with mild symptoms.

Symptoms may not only affect the fingers, but also the toes, lips, ears and nose.

An episode may range from a few minutes to a number of hours.

Breastfeeding - Raynaud's-type symptoms may occur during breastfeeding; the nipples will turn white and become extremely painful.

What are the risk factors?

A risk factor is something which increases the likelihood of developing a condition or disease. For example, obesity significantly raises the risk of developing diabetes type 2. Therefore, obesity is a risk factor for diabetes type 2.

• Gender. The phenomenon is more common in women than men; the Framingham Study found that 5% of men and 8% of women suffer from it.
• Age. Although any age group can be affected, middle-aged and elderly individuals have a higher risk, compared to young people.
• Geography. A significantly higher percentage of adults in, for example, Alaska suffer from the phenomenon compared to individuals in Florida.
• Genes. A significant number of individuals with Raynaud's disease have a parent or sibling who also has it.
• Underlying diseases - people with some other underlying diseases have a much higher risk of developing the phenomenon, such as people:
  
  
  • with connective tissue disorders, including scleroderma, systemic lupus erythematosus, rheumatoid arthritis, Sjögren's syndrome, dermatomyositis, polymyositis, mixed connective tissue disease, cold agglutinin disease, and Ehlers-Danlos Syndrome.
  • with eating disorders, such as anorexia nervosa.
  • with obstructive disorders, such as atherosclerosis, Buerger's disease, Takayasu's arteritis, subclavian aneurysms, and thoracic outlet syndrome.
  • on certain medications, such as beta-blockers, cytotoxic drugs - particularly chemotherapeutics and most especially bleomycin, ciclosporin, ergotamine, sulfasalazine, and anthrax vaccines whose primary ingredient is the Anthrax Protective Antigen.
  • some other conditions, such as hypothyroidism, cryoglobulinemia, malignancy, reflex sympathetic dystrophy, carpal tunnel syndrome, and Magnesium Deficiency Erythromelalgia

What causes Raynaud's disease?

When the body is exposed to the cold, heat is lost through the extremities (fingers and toes) as capillaries (small blood vessels) under the skin constrict - become narrower, resulting in less blood getting to tissue in that area. For people with Raynaud's disease, the narrowing is more severe, and the consequent blood supply is much less than other people. If the blood supply is very low, that part of the body experiences an abnormal drop in temperature - hence the sensation of cold fingers.

For some people it needs to get really cold for symptoms to appear, while for others even a slight drop in temperature can trigger symptoms.

Stress, specifically emotional stress, which includes anxiety or anger can also trigger symptoms.

In secondary Raynaud's, where there is an underlying condition/disease, we know that the cause is that condition. In primary Raynaud's, nobody really knows why the blood vessels narrow so much.

Some occupations - there may be a link between repetitive movements and Raynaud's type symptoms, as may happen with a typist whose fingertips receive little blows for prolonged periods. Certain occupations may make individuals more susceptible, especially those where vibrating equipment is used, such as hammer drills, chainsaws or hedge trimmers (vibration white finger). Some people who work in the plastics industry develop Raynaud's type symptoms.

Smoking - smoking causes the blood vessels to narrow, increasing the risk of developing Raynaud's disese.

What are the complications of Raynaud´s disease?

A combination of thickening blood vessel walls and narrowing can lead to permanent reduction of blood flow to susceptible areas. If blood flow is seriously impaired there is a risk of finger or toe deformity, and even gangrene.

Some people with Raynaud's syndrome go on to develop scleroderma, where scar tissue (fibrosis) forms in the skin and sometimes other organs of the body.

How is Raynaud´s disease diagnosed?

Most doctors, after a careful examination, will be able to determine whether the patient has the primary or secondary form of Raynaud's. Possible secondary causes need to be identified or excluded. The following diagnostic tests may be ordered:

• Digital artery pressure - the pressure of the arteries of the fingers are measured before and after they are cooled. A drop of 15mmHg or more allows for a positive diagnosis.
• Blood test - to determine blood count (may reveal anemia or renal failure). Urea and electrolyte levels may also be tested to rule out a kidney problem (renal impairment).
• Thyroid function test - to rule out hypothyroidism (underactive thyroid gland)
• An auto-antibody screen - to test for rheumatoid factor, Erythrocyte sedimentation rate, or C-reactive protein. These may reveal either an inflammatory process or some illnesses.
• Nail fold vasculature capillaroscopy - a fold of hard skin overlapping the base and sides of a finger/toe nail is examined under a microscope and the tiny blood vessels are observed. This test can help determine whether the patient has the primary or secondary form of the disease.

What is the treatment for Raynaud´s disease?

Treatment options depend on the type (secondary or primary), and the severity of symptoms. Treatment for primary Raynaud's concentrates primarily on avoiding triggers.

If symptoms are mild, most patients find that simply avoiding the triggers, such as staying warm, learning to relax (to avoid stress, anxiety and anger), and avoiding vibrating machines is all they need to do. Smokers should seriously consider giving up.

Avoiding caffeine, stimulants and substances that cause the blood vessels to constrict (vasoconstrictors) may help alleviate symptoms.

If the fingers become extremely white, run tepid to slightly warm water over them and massage them gently. If no warm water is available, place your fingers under your arms, crotch, or even in your mouth. Keep the affected digits warm until their normal color is restored.

Medications

• Nifedipine - this medication, a dihydropyridine calcium channel blocker, rapidly lowers blood pressure and opens up the capillaries (tiny blood vessels); this helps speed up the return of proper bloodflow to affected areas. Patients should not drink grapefruit juice while on this medication.
• Angiotensin blocking vasodilators - angiotensin is a protein that makes our blood vessels narrow (constrict) and drives up blood pressure. A vasodilator is anything which opens up (widens) blood vessels. An angiotensin blocking vasodilator is a medication that blocks angiotensin and widens the blood vessels.
• Iloprost - if symptoms are severe and other therapies were not effective, this medication may be administered by intravenous infusion (through a drip). Iloprost widens the blood vessels, helping proper blood flow reach the affected areas.

Counseling - if the patient has the primary form of Raynaud's and emotional stress is identified as one of the triggers, counseling may help control stress, anger, frustration and anxiety. In some cases the patient may be prescribed an antidepressant.

Surgery - in extreme cases that have not responded to other therapies, some nerves in the affected area may be cut. This procedure is known as sympathectomy.

Written by Christian Nordqvist
Copyright: Medical News Today
Not to be reproduced without permission of Medical News Today

• Additional
• References
• Citations