Causes and Treatment of Raynaud's Syndrome

Raynaud's syndrome is a phenomenon in which a person's fingers, and sometimes toes, turn blue or white when exposed to cold and bright red upon rewarming. It is caused by sudden and excessive vasoconstriction (narrowing of blood vessels) that can last for seconds to hours. Moments of stress can also trigger symptoms in some people.

Raynaud's syndrome can occur on its own or with diseases like scleroderma, lupus, and others. Raynaud's syndrome may be treated by avoiding cold triggers or resolving the underlying condition that causes it. The vast majority of cases are not serious.

This article describes the symptoms, causes, and treatment of Raynaud's syndrome and offers self-help tips so you can better cope with this harmless but sometimes aggravating symptom.

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Types of Raynaud's Syndrome

There are two types of Raynaud's syndrome: the primary form which is idiopathic (occurring for no known cause) and the secondary form associated with an underlying condition.

Primary Raynaud's Disease

Primary Raynaud's is the type that occurs on its own with no underlying disease. It is more common in young females, with a peak incidence between ages 15 and 25.

The condition is usually mild and can usually be controlled by lifestyle measures. Some people refer to it as a "cold allergy," although it has no association with the immune reaction that causes allergies.

Secondary Raynaud's Phenomenon

Secondary Raynaud's syndrome is caused by an underlying disease or condition. Connective tissue disorders are the most common cause, but certain vascular or hormonal diseases are also linked to the condition. Some medications can do the same.

Connective Tissue/Autoimmune Diseases

• Scleroderma
• Mixed connective tissue disease
• Dermatomyositis
• Polymyositis
• Systemic lupus erythematosus (lupus)
• Rheumatoid arthritis

Other Medical Conditions

• Hypothyroidism
• Pulmonary hypertension
• Fibromyalgia
• Chronic fatigue syndrome
• Atherosclerosis
• Carpal tunnel syndrome
• Lyme disease
• Multiple sclerosis
• Previous hand surgery or injury
• A history of frostbite
• Occupations that involve vibration (like jackhammers) or prolonged exposure to cold (like frozen food packers)

Medications

• Beta-blockers
• Some chemotherapy drugs (like cisplatin, vinblastine, bleomycin)
• Some migraine medications (like ergot derivatives and sumatriptan)
• Pseudoephedrine used in OTC allergy formulations
• Stimulants (like amphetamine)
• Estrogen-containing medications

Signs and Symptoms of Raynaud's Syndrome

Primary and secondary Raynaud's syndrome is characterized by the constriction of small blood vessels—usually on both sides of the body—in the fingers or toe. The condition may also affect the tip of the nose, earlobes, skin over the kneecap, or nipples in some people.

Symptoms of Raynaud's disease occur in two stages:

1. When exposed to cold, the skin will turn bluish or white and there will be localized pain, coldness, or numbness.
2. When the skin is reheated, it will turn bright red and there will be tingling, swelling, throbbing, or a painful pins-and-needles sensation.

The symptoms can range from barely noticeable to severe, interfering with a person's quality of life.

On rare occasions, severe vasoconstriction has led to the formation of skin ulcers or gangrene due to the protracted loss of blood flow.

What is the Main Cause of Raynaud's Syndrome?

Raynaud's syndrome affects roughly 5% of the population in the United States to varying degrees. It is more common in colder climates and those with a family history of the condition. It is also more common in females than males, particularly females in their childbearing years.

Raynaud's disease occurs when vasoconstriction, an otherwise normal process that regulates blood circulation and body temperature, is overreactive or exaggerated.

In response to cold, blood vessels will spontaneously narrow, causing the sudden deprivation of blood to the fingers. The trigger localized pain and the sudden whitening (pallor) or bluish discoloration of the skin (cyanosis).

When the fingers are reheated, the dilation (widening) of blood vessels is also exaggerated, causing pain and redness (rubor) as blood rushes back into the vessels.

The mechanism for the symptoms varies by whether the condition is primary or secondary:

• With primary Raynaud's syndrome, vasoconstriction is thought to be due to hypersensitivity of the sympathetic nervous system which regulates involuntary functions like blood circulation. Given that females are more commonly affected, hormones may also contribute as some directly or indirectly influence the sympathetic nervous system.
• With secondary Raynaud's phenomenon, underlying diseases can cause changes to how blood flows to the blood vessels of the fingers or toes. Connective tissue diseases directly affect blood vessel walls, while vascular conditions (like atherosclerosis) affect circulation. Certain drugs and hormonal conditions (like hypothyroidism) affect hormones involved in vasoconstriction.
  

How Raynaud's Syndrome Is Diagnosed

A diagnosis of Raynaud's syndrome starts with a review of your medical history, including your symptoms, any medications you take, and any medical condition you have. This is accompanied by a physical exam and blood tests.

As part of the physical workup, certain in-office tests can determine if primary or secondary Raynaud's syndrome is involved, such as:

• Nail fold capillaroscopy: This involves a special camera (called a dermatoscope) to view tiny blood vessels (capillaries) at the base of the nail. Smaller-than-expected capillaries are a sign of connective tissue disease.
• Cold stimulation test: This involves the placement of heat sensors on your fingers. When your fingers are dipped and removed from an ice-water bath, the sensors can measure how quickly your fingers warm up and return to normal.

Blood tests commonly performed in the diagnosis of Raynaud's syndrome include:

• Antinuclear antibody test (ANA) used to detect autoimmune/connective tissue diseases
• Thyroid hormone tests, used to detect if hypothyroidism
• High-sensitivity CRP (hs-CRP), used to detect inflammation associated with atherosclerosis
• Erythrocyte sedimentation (ESR), used to detect generalized inflammation seen with autoimmune/connective tissue diseases

Other tests or procedures may be ordered to further narrow the possible causes.

Diagnostic Criteria

A diagnosis of Raynaud's syndrome may seem obvious, but certain criteria must be met to determine if the condition is primary or secondary.

Diagnostic criteria for primary Raynaud's syndrome include:

• Episodes of pallor or cyanosis on the fingers or toes triggered by cold or stress that occurs over a period of at least two years
• Symmetric symptoms
• The absence of a known underlying cause
• Normal ESR results
• Normal findings on a nail fold capillaroscopy

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

• Episodes of pallor or cyanosis on the fingers or toes triggered by cold or stress that occurs over a period of at least two years
  
• A known underlying condition
• An abnormal nail fold capillaroscopy
• Positive ANA results
• High ESR results
• Scars, ulcers, or gangrene of the fingers or toes
  

Can Raynaud's Syndrome Be Treated?

Generally, Raynaud's disease is not treated, although steps are taken to avoid triggers such as cold or stress. If secondary Raynaud's syndrome is involved, resolving the underlying condition or changing medications can usually resolve the symptoms.

With that said, certain medications or procedures can be used if the symptoms are so severe as to undermine your quality of life.

Lifestyle Modifications

Raynaud's syndrome can often be managed with relatively simple lifestyle changes. Here are eight to consider:

• Protect your hands with mittens or gloves and your feet with thick socks when out in the cold.
• Wear gloves when handling cold items.
• Use insulated neoprene covers when drinking out of a can or bottle.
• Warm your car before leaving home, and consider investing in a remote car starter.
• Wear shoes rather than going barefoot.
• Avoid tight rings, shoes, watchbands, and anything that constricts circulation to your hands or feet.
• Avoid medications linked to Raynaud's, including cold medications like Sudafed (pseudoephedrine).
• Practice stress reduction techniques like yoga, meditation, deep breathing, and guided imagery.
  

Medications

Medications may be used if lifestyle measures are inadequate. These are most often used in people with secondary Raynaud's syndrome, although some may benefit those with secondary Raynaud's.

Calcium channel blockers, which work to dilate blood vessels, are some of the safest and most helpful medications for Raynaud's. These include Procardia (nifedipine), Norvasc (amlodipine), and Cardizem or Dilacor (diltiazem).

Calcium channel blockers may also be beneficial for people with primary Raynaud's disease. For some, the drugs may only be needed during the winter and can be stopped during the summer.

Other medications used to ease symptoms of Raynaud's include:

• Alpha-blockers like Minipress (prazosin), Cardura (doxazosin), and Hytrin (terazosin)
• Angiotensin-receptor blockers like Hyzaar (losartan)
• Blood thinners like aspirin or Persantine (dipyridamole)
• Phosphodiesterase-5 inhibitors like Viagra (sildenafil), Cialis (tadalafil), and Levitra (vardenafil).
• Selective serotonin reuptake inhibitors like Prozac (fluoxetine)
• Oral prostaglandins like Cytotec (misoprostol)
• Topical nitroglycerin, available as an ointment, paste, gel, or patch

The endothelin receptor inhibitor Tracleer (bosentan) paired with Ventavis (iloprost) has proven beneficial to people with severe Raynaud's caused by scleroderma.

Procedures

Procedures are used less frequently and only used when lifestyle or drug options fail. These include:

• Sympathectomy: This is a surgical procedure in which the sympathetic nerves that cause the vasoconstriction are cut. While effective, improvements are often short-lived.
• Local injections: A local injection of Botox or a local anesthetic may interrupt nerve signals that cause vasoconstriction.
  

Summary

Raynaud's syndrome is a generally harmless phenomenon where your fingers or toes turn pale and painful when exposed to cold and red and painful when reheated. Stress can also cause Raynaud's.

There is a primary form of Raynaud's that occurs for no reason and secondary forms of Raynaud's linked to autoimmune, connective, vascular, or hormonal diseases as well as certain drugs.

Primary Raynaud's syndrome is mainly treated by avoiding cold and stress triggers. Secondary Raynaud's syndrome is often resolved by treating the underlying condition.