What is Raynaud’s Disease?
Raynaud’s Disease (also called Raynaud’s Phenomenon) is a medical condition. It is named after the French physician, Maurice Raynaud, who first described it. Although the fingers of healthy individuals may become pale in response to severe colds, the effect is exaggerated in individuals with Raynaud’s symptoms. Even mild cold exposure can cause significant symptoms. 80% of sufferers are women, and as many as 5% of the U.S. population has Raynaud’s disease.
Primary “Raynaud’s Disease” refers to vasospasm (severe, temporary narrowing of the arteries) that happens with cold or stress without any other related medical condition. Some people have other medical illnesses, such as scleroderma or systemic lupus erythematosus. In these cases, “Raynaud’s Phenomenon” is used to describe vasospasm of the digits in response to cold. The cause is not known.
There are two types of Raynaud’s phenomenon.
- Primary Raynaud’s phenomenon occurs for an unknown reason. It is the more common form of Reynaud’s phenomenon.
Raynaud phenomenon is an episodic, reversible vasospasm of the peripheral arteries, usually digital. It causes pallor followed by cyanosis and/or redness, often with pain and, at times, paresthesia. On rare occasions, it can lead to ulceration of the fingers and toes and, in some cases, of the ears or nose. This review focuses on the primary (idiopathic) Raynaud phenomenon occurring in the absence of an underlying disease.
- Prevalence, which varies by sex and country, is around 3% to 5% in most population studies, 80% to 90% of which is a primary Raynaud phenomenon; prevalence is slightly higher in women than in men.
- Attacks may last from several minutes to a few hours, and persons with initial long-term idiopathic Raynaud phenomenon can later go on to display features of underlying disorders such as systemic sclerosis.
Nifedipine seems to reduce the frequency and severity of Raynaud attacks, although it is associated with high rates of adverse effects such as tachycardia, headache, and flushing.
We found no evidence of sufficient quality to judge the effectiveness of amlodipine or diltiazem in treating the primary Raynaud phenomenon.
Nicardipine may successfully treat the primary Raynaud phenomenon, but we found no studies large enough to draw firm conclusions.
- Secondary Raynaud’s phenomenon is caused by another health condition. Secondary Raynaud’s phenomenon is less common but more serious than the primary form of the disease.
What Is Secondary Raynaud’s?
Secondary Raynaud’s is Raynaud’s whereby the cause is caused by an underlying medical condition such as scleroderma, lupus, arthritis, heart disease, carpal tunnel syndrome, or frostbite. Other factors can also come into play, such as smoking or your line of work. For example, workers who use mechanical machinery and tools without the proper PPE can suffer from a vibration-caused form of Raynaud’s called Hand Arm Vibration Syndrome or HAVS.
The second most common place to suffer from Raynaud’s is on your feet. Thankfully, special thermal socks can be used to keep your feet warm even in freezing temperatures and you can even buy warming insoles that prevent your feet from getting cold in the first place.
Lastly, getting Raynaud’s toes can be common as well. Should you get your shoes wet, then often your toes will be the first to succumb. Cold toes are among the most difficult parts of your body to keep warm.
Where Can I Get Raynaud’s?
Raynaud’s is common in the extremities of your body. The most common place is in your hands, where your fingers can quickly feel the cold. Changes in temperature can lead to your hands turning white, which can be countered by using gloves, hand warmers, and more.
Attacks usually happen when you are cold or feeling stressed. During an attack, your fingers and toes may feel very cold or numb.
Episodes are typically triggered by cold or emotional stress. Primary Raynaud’s, also known as idiopathic, means that it is spontaneous, of unknown cause, and unrelated to another disease. Secondary Raynaud’s occurs as a result of another condition and has an older age at onset; episodes are intensely painful and can be asymmetric and associated with skin lesions. Secondary Raynaud’s can occur due to a connective-tissue disorder such as scleroderma or lupus, injuries to the hands, prolonged vibration, smoking, thyroid problems, and certain medications, such as birth control pills. Diagnosis is typically based on the symptoms.
The primary treatment is avoiding the cold. Other measures include the discontinuation of nicotine or stimulant use. Medications for the treatment of cases that do not improve include calcium channel blockers and iloprost. Little evidence supports alternative medicine. Severe disease may in rare cases lead to complications, specifically skin sores or gangrene.
About 4% of people have the condition. The onset of the primary form is typically between ages 15 and 30 and occurs more frequently in females. The secondary form usually affects older people. Both forms are more common in cold climates.
During attacks, the small vessels that supply blood to the skin in the hands, and sometimes the toes, nose, or ears constrict, resulting in numbness and a pale or bluish tint.
The condition can cause localized pain, discoloration (paleness), and sensations of cold and/or numbness.
When exposed to cold temperatures, the blood supply to the fingers or toes, and in some cases the nose or earlobes, is markedly reduced; the skin turns pale or white (called pallor) and becomes cold and numb. These events are episodic, and when the episode subsides or the area is warmed, the blood flow returns and the skin color first turns red (rubor), and then back to normal, often accompanied by swelling, tingling, and a painful “pins and needles” sensation. All three color changes are observed in classic Raynaud’s. However, not all patients see all of the aforementioned color changes in all episodes, especially in milder cases of the condition. The red flush is due to reactive hyperemia of the areas deprived of blood flow.
In pregnancy, this sign normally disappears due to increased surface blood flow. Raynaud has occurred in breastfeeding mothers, causing nipples to turn white and painful.
Distinguishing Raynaud’s disease (primary Raynaud’s) from Raynaud’s phenomenon (secondary Raynaud’s) is important. Looking for signs of arthritis or vasculitis, as well as a number of laboratory tests, may separate them. Nail fold capillary examination or “capillaroscopy” is one of the most sensitive methods to diagnose RS with connective tissue disorders, i.e. distinguish a secondary from a primary form objectively.
If suspected to be secondary to systemic sclerosis, one tool which may help aid in the prediction of systemic sclerosis is thermography.
A careful medical history will seek to identify or exclude possible secondary causes.
- Digital artery pressures are measured in the arteries of the fingers before and after the hands have been cooled. A decrease of at least 15 mmHg is diagnostic (positive).
- Doppler ultrasound to assess blood flow
- Full blood count may reveal normocytic anemia suggesting the anemia of chronic disease or kidney failure.
- Blood tests for urea and electrolytes may reveal kidney impairment.
- Thyroid function tests may reveal hypothyroidism.
- Tests for rheumatoid factor, erythrocyte sedimentation rate, C-reactive protein, and autoantibody screening may reveal specific causative illnesses or an inflammatory process. Anti-centromere antibodies are common in limited systemic sclerosis (CREST syndrome).
- Nail fold vasculature (capillaroscopy) can be examined under a microscope.
To aid in the diagnosis of Raynaud’s phenomenon, multiple sets of diagnostic criteria have been proposed. Table 1 below provides a summary of these various diagnostic criteria.
Recently, International Consensus Criteria were developed for the diagnosis of primary Raynaud’s phenomenon by a panel of experts in the fields of rheumatology and dermatology.
Secondary Raynaud’s is managed primarily by treating the underlying cause, and as primary Raynaud’s, avoiding triggers, such as cold, emotional, and environmental stress, vibrations, and repetitive motions, and avoiding smoking (including passive smoking) and sympathomimetic drugs.
Medications can be helpful for moderate or severe diseases.
- Vasodilators – calcium channel blockers, such as dihydropyridines nifedipine or amlodipine, preferably slow-release preparations – are often first-line treatments. They have the common side effects of headache, flushing, and ankle edema, but these are not typically of sufficient severity to require cessation of treatment. The limited evidence available shows that calcium-channel blockers are only slightly effective in reducing how often the attacks happen. Although, other studies also reveal that CCBs may be effective at decreasing the severity of attacks, pain, and disability associated with Raynaud’s phenomenon. People whose disease is secondary to erythromelalgia often cannot use vasodilators for therapy, as they trigger ‘flares’ causing the extremities to become burning red due to too much blood supply.
- People with severe disease prone to ulceration or large artery thrombotic events may be prescribed aspirin.
- Sympatholytic agents, such as the alpha-adrenergic blocker prazosin, may provide temporary relief to secondary Raynaud’s phenomenon.
- Losartan can, and topical nitrates may reduce the severity and frequency of attacks, and the phosphodiesterase inhibitors sildenafil and tadalafil may reduce their severity.
- Angiotensin receptor blockers or ACE inhibitors may aid blood flow to the fingers, and some evidence shows that angiotensin receptor blockers (often losartan) reduce the frequency and severity of attacks, and possibly better than nifedipine.
- The prostaglandin iloprost is used to manage critical ischemia and pulmonary hypertension in Raynaud’s phenomenon, and the endothelin receptor antagonist bosentan is used to manage severe pulmonary hypertension and prevent finger ulcers in scleroderma.
- Statins have a protective effect on blood vessels, and SSRIs such as fluoxetine may help symptoms, but the data is weak.
- PDE5 inhibitors are used off-label to treat severe ischemia and ulcers in fingers and toes for people with secondary Raynaud’s phenomenon; as of 2016, their role more generally in Raynaud’s was not clear.
- In severe cases, an endoscopic thoracic sympathectomy procedure can be performed. Here, the nerves that signal the blood vessels of the fingertips to constrict are surgically cut. Microvascular surgery of the affected areas is another possible therapy, but this procedure should be considered a last resort.
- A more recent treatment for severe Raynaud’s is the use of botulinum toxin. The 2009 article studied 19 patients ranging in age from 15 to 72 years with severe Raynaud’s phenomenon of which 16 patients (84%) reported pain reduction at rest; 13 patients reported immediate pain relief, and three more had gradual pain reduction over 1–2 months. All 13 patients with chronic finger ulcers healed within 60 days. Only 21% of the patients required repeated injections. A 2007 article describes similar improvement in a series of 11 patients. All patients had significant relief from pain.
Evidence does not support the use of alternative medicine, including acupuncture and laser therapy.
The prognosis of primary Raynaud syndrome is often very favorable, with no mortality and little morbidity. However, a minority develops gangrene. The prognosis of secondary Raynaud is dependent on the underlying disease, and how effective blood flow-restoring maneuvers are.
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