# Raynaud's syndrome | Symptoms, Causes, Tests and Treatment | OSANG

> Raynaud's syndrome occurs in approximately 3-5% of the world's population and is common in young women. Primary Raynaud's phenomenon (Raynaud's disease) occurs without a…

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- Organization: OSANG Neurosurgery

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Autonomic Medicine

## Raynaud's syndrome

Raynaud's Syndrome · I73.0

Raynaud's syndrome is an autonomic nerve-vascular reaction abnormality in which excessive vasospasm occurs in peripheral blood vessels (mainly fingers and toes) due to cold or emotional stress, causing skin color to change to triphasic from pale to blue to red, and causing numbness and pain.

## At a glance

Raynaud's syndrome occurs in approximately 3-5% of the world's population and is common in young women. Primary Raynaud's phenomenon (Raynaud's disease) occurs without an underlying disease and has a good prognosis. Secondary Raynaud's phenomenon is caused by autoimmune diseases such as systemic sclerosis, lupus, and rheumatoid arthritis, exposure to vibration, or certain drugs, and carries a risk of ischemic complications. Sympathetic overactivity and vascular endothelial dysfunction are the main mechanisms, and calcium channel blockers are used as first-line treatments.

- 01Definition and Overview

- 02Triphasic skin color changes

- 03Mechanism of occurrence

- 04Distinguish between primary and secondary

- 05treatment

## Definition and Overview

Raynaud's syndrome is a condition in which excessive vasospasm occurs in peripheral arteries and arterioles in response to cold or emotional stress. The fingers are most commonly affected, but the toes, ears, nose, and lips may also appear.

It occurs in approximately 3-5% of the world's population, and is approximately 5-9 times more common in women than in men. It is especially common in young women aged 15 to 30. It is divided into primary Raynaud's phenomenon (formerly 'Raynaud's disease') and secondary Raynaud's phenomenon with an underlying disease.

## Triphasic skin color changes

A triphasic color change in skin color that occurs during Raynaud's attack is characteristic.

Pallor: When local blood flow is interrupted due to vascular spasm, the skin turns white. Cyanosis: Stagnant blood turns blue as oxygen saturation decreases. Redness (erythema/rubor): As blood vessel spasm is relieved, blood flows rapidly and becomes red. The recovery period may be accompanied by burning pain.

The three stages may not be clearly visible in all patients, and biphasic changes directly leading to redness after pallor are also common.

## Mechanism of occurrence

The mechanism of occurrence of Raynaud's syndrome is complex.

Sympathetic nerve hyperactivity and alpha-2 receptor hypersensitivity are the key. When cold is stimulated, norepinephrine is secreted from sympathetic nerve terminals, and alpha-2C receptors in vascular smooth muscles recognize this and constrict blood vessels. In Raynaud's patients, this receptor is hypersensitive or has increased expression in blood vessels. The mechanism by which alpha-2C receptor movement to the cell surface increases in a low-temperature environment was also confirmed.

Vascular endothelial dysfunction is also involved. The production of nitric oxide (NO) and prostacyclin, which induce normal vasodilation, decreases, and the secretion of vasoconstrictor substances (endothelin-1) increases, resulting in a decrease in vasodilation ability.

In secondary Raynaud's phenomenon, vascular structural damage caused by an autoimmune mechanism additionally occurs. Systemic sclerosis is accompanied by structural vascular stenosis in addition to functional vasospasm due to damage to vascular endothelial cells and fibrosis of the vascular wall.

## Distinguish between primary and secondary

Diseases that cause secondary Raynaud's phenomenon include connective tissue diseases (systemic sclerosis, systemic lupus erythematosus, mixed connective tissue disease, rheumatoid arthritis, Sjögren's syndrome), occupational factors (use of vibrating tools, repetitive trauma), drugs (beta blockers, ergotamine, anticancer drugs), vascular occlusive diseases (thrombosis, arteriosclerosis), endocrine diseases (hypothyroidism), and neurological diseases.

Tests that may be helpful in differential diagnosis include nailfold capillaroscopy, antinuclear antibody (ANA), anti-CCP antibody, complement, rheumatoid factor, and complete blood count. If abnormal capillaries (dilatation, loss, bleeding) are observed under a capillary microscope, it suggests an association with connective tissue disease.

## treatment

### Non-pharmacological treatment

Insulation is the most basic. Minimize exposure to cold environments and use thick gloves and hot packs. Keeping your whole body warm is more effective than keeping just your fingers warm. Quitting smoking is important. Nicotine constricts blood vessels, causing or worsening Raynaud's attacks. Relaxation therapy, biofeedback, and cognitive behavioral therapy for stress management may be helpful.

### medication

Calcium channel blockers are the first-line drugs. In a meta-analysis, long-acting dihydropyridine calcium channel blockers such as amlodipine and nifedipine were reported to reduce the frequency of Raynaud's attacks by about 30-40%.

If you have finger ulcers or are at high risk for secondary Raynaud's phenomenon, phosphodiesterase-5 inhibitors (sildenafil), intravenous prostacyclin (iloprost), and endothelin receptor blockers (bosentan) are used.

Sympathetic nerve blockade (stellate ganglion blockade, intrafatty sympathetic nerve blockade) is attempted to control vasospasm in severe secondary Raynaud's phenomenon.

## Frequently asked questions

### Q01What is Raynaud's Syndrome?

This is a disease in which the blood vessels in the fingers (or toes) contract excessively and blood supply is reduced during cold weather or emotional stress. As the skin color changes from pale to blue to red, numbness and pain appear and then recover when warmth is applied. Unlike simply cold or numb fingers, it is characterized by a distinct color change. It is mainly common in young women, and most cases are primary with no major health problems, but in some cases, it is accompanied by an underlying disease.

### Q02How does Raynaud's syndrome relate to the autonomic nervous system?

Excessive activation of the sympathetic nerves, which constrict blood vessels, is the core mechanism of Raynaud's syndrome. Blood vessel constriction occurs much stronger than normal in response to cold or stress stimuli. Norepinephrine secreted from sympathetic nerve terminals stimulates alpha-2 receptors in vascular smooth muscles, and in Raynaud's patients, these receptors are hypersensitive or overexpressed. Assessment of autonomic function may reveal a pattern of sympathetic overactivity.

### Q03How to distinguish between primary and secondary Raynaud's phenomenon?

Primary (Raynaud's disease) occurs without underlying disease and has a good prognosis. Most cases occur before the age of 40, are bilaterally symmetrical, have no ischemic complications (ulcers, gangrene), are negative for antinuclear antibodies (ANA), and are characterized by normal findings on capillary microscopy. Secondary is the presence of an underlying disease and is common in systemic sclerosis, lupus, rheumatoid arthritis, and Sjögren's syndrome. It is distinguished by its onset at a late age, asymmetry, accompanying finger ulcers, positive antinuclear antibodies, and capillary abnormalities.

### Q04How is Raynaud's syndrome treated?

Lifestyle management is basic. Staying warm (thick gloves, hot packs), quitting smoking, managing stress, and avoiding excessive caffeine intake are helpful. If lifestyle management does not provide sufficient control, calcium channel blockers (amlodipine, nifedipine) are used, and studies have shown that the number of attacks is reduced in about one third. In secondary cases, phosphodiesterase inhibitors such as sildenafil or prostacyclins such as iloprost may be used to prevent finger ulcers.

### Q05Is Raynaud's syndrome a dangerous disease?

If you only have primary Raynaud's phenomenon, it may cause discomfort in your daily life, but it rarely leads to finger ulcers or tissue damage, so the risk is low. However, secondary Raynaud's phenomenon depends on the underlying disease. In particular, patients with systemic sclerosis who are accompanied by Raynaud's phenomenon may develop finger ischemic ulcers, gangrene, and non-healing wounds, so active treatment is required. If you are experiencing Raynaud's symptoms for the first time, you must see a specialist to rule out secondary causes.

### Q06Does stress make Raynaud's syndrome worse?

Yes, psychological stress is an important trigger for Raynaud's attacks. During stress, sympathetic nerve activity increases and peripheral vasoconstriction worsens. In fact, Raynaud's attacks caused by emotional stress produce the same vascular response as when cold stimulation is applied to the fingers. Studies have shown that stress management, relaxation techniques, and biofeedback may help reduce the frequency of Raynaud's attacks.

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