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What is Raynaud’s phenomenon?

In 1862, Auguste-Maurice Raynaud in his thesis “Local asphyxia and symmetrical gangrene of the
extremities” described for the first time color changes of the hands and feet triggered by exposure to
cold temperatures. Today we call this exaggerated response to cold “Raynaud’s phenomenon” in
memory of this French physician.
Raynaud’s (ray-NODES) phenomenon is characterized by development of episodes of pallor (white
fingers or toes), or cyanosis (blue discoloration) in response to cold exposure or emotional stress.
The pallor is caused by spasm of blood vessels to the fingers (vasoconstriction) that results in a
temporary cut-off of blood supply to the skin, while cyanosis is caused by extraction of oxygen from
stagnant or sluggish flowing blood. Upon re-warming, the blood flow is restored and the skin appears
reddened or blushed. Pallor is the most important physical sign. In Raynaud’s phenomenon it is
sharply demarcated at the ends of fingers (figure) and toes, and not mottled irregularly. Tingling,
numbness, and pain can be associated with the color changes. Episodes can last from minutes to
sometimes hours, and can affect some or all fingers, and sometimes the palms too. Rarely,
Raynaud’s can also affect other body parts such as ears, nose, etc. Usually episodes occur as
sudden attacks, often triggered by rapid changes in the ambient temperature.

Primary Raynaud’s phenomenon is not associated with any underlying disease. It occurs in 5-10% of
the general population. It is more common among young women and in countries with cold climates.
It is believed that in some individuals the smooth muscle of blood vessels are very sensitive and
have an exaggerated response to cold temperatures, causing spasm of the blood vessels and
leading to primary Raynaud’s phenomenon. The condition may be familial. Recent studies find that
about 30% of people with primary Raynaud’s phenomenon have a first degree relative with the same
condition.
Secondary Raynaud’s phenomenon occurs in patients with a defined cause or an associated
disease:

1. Rheumatic diseases Scleroderma, systemic lupus erythematosus, mixed connective

tissue disease, Sjögren’s syndrome, dermatomyositis, etc.

2. Vibrating tools Jackhammer, power drill, etc (vibration white finger)

3. Drugs Sympathomimetics (antihistamines, ephedrine, epinephrine,

phenylpropanolamine)
Migraine remedies (ergot alkaloids, triptans)
Some chemotherapeutic agents (bleomycin)

4. Chemicals Nicotine, cocaine, vinyl chloride (occupational exposure)

5. Occlusive vascular disease Peripheral vascular disease, Buerger’s disease, thoracic outlet
syndrome
6. Hyperviscosity Cryoglobulinemia, paraproteinemias

7. Metabolic diseases Hypothyroidism

How are patients with Raynaud’s phenomenon evaluated?

Raynaud’s phenomenon is a clinical diagnosis, suggested by a history of cold sensitivity, with the
associated typical triphasic color changes (white, blue, and red) of the skin. During presentation a
complete history and physical examination is necessary to look for any underlying cause for the
attacks. Presence of swelling of hands and fingers, tightening of skin and history of fingertip ulcers
are warning signs of scleroderma or mixed connective tissue disease. One special test is nail fold
capillaroscopy, where a doctor puts a drop of oil on the patients’ nail fold and examines the area
under a microscope to look for any changes in the capillary (small blood vessel) pattern. Enlarged,
dilated or absent (dropout) nail fold capillaries are seen in patients with scleroderma,
dermatomyositis and other connective tissue diseases. Blood tests (such as an ANA test) are
performed if an underlying connective tissue disease is suspected.
How is Raynaud’s phenomenon treated?
The goals of treatment are (1) to reduce the number and severity of the attacks, and (2) to prevent
tissue damage.
Advice given to all patients includes avoidance of cold temperatures and stress management.
Treatment includes methods to avoid the common provoking and aggravating factors for Raynaud’s
attacks.
Keeping the whole body warm (not just the fingers) is very important. This is best achieved by
wearing layers of clothing and also covering the head with a hat. Sudden temperature changes,
particularly wet, cold, and windy weather, are bad for Raynaud’s sufferers. Wearing warm gloves or
mittens can be useful. Chemical warmers placed in pockets are also quite effective in aborting
attacks.
Smoking can worsen attacks, as nicotine causes spasm of blood vessels in the fingers and toes.
Also certain drugs (which cause vasospasm) should be avoided – e.g. over the counter cold
remedies containing sympathomimetics (e.g. Sudafed®), clonidine, migraine medications containing
ergot alkaloids. Estrogens and non-selective beta blockers (like propranolol) can also aggravate
Raynaud’s attacks.
Drug therapy is not indicated in every case. In primary Raynaud’s phenomenon, the attacks are
usually mild and do not cause any tissue damage (leading to digital ulcers or gangrene). Hence non-
drug therapy (as outlined above) is recommended. Drug therapy (as outlined below) may be
necessary if the attacks are severe, alter the quality of life, and compromise the ability to perform the
activities of daily living.
Drug therapy is usually recommended in patients with secondary Raynaud’s phenomenon who have
severe attacks and if there is risk of tissue damage. The most common medications are calcium
channel blockers such as nifedipine (Procardia XL®) or Amlodipine (Norvasc®). Other drugs that can
be used include alpha-adrenergic blockers (Prazosin or Tamsulosin), or local application of
nitroglycerine patches or ointment. Other drugs that are useful in some cases include angiotensin
receptor blockers (e.g. Losartan), phosphodiesterase 5 inhibitors [e.g. sildenafil (Viagra®)], and
selective serotonin reuptake inhibitors (e.g. Fluoxetine). Anti-platelet drugs like aspirin or clopidogrel
(Plavix®) are often added.
For prevention of ischemic digital ulcers in scleroderma patients, recent studies have shown that
endothelin receptor antagonists (e.g. bosentan (Tracleer®)) are showing promise. For fingers with
severe ulceration or impending gangrene, hospitalization for a trial of a prostacyclin analog
intravenously (e.g. epoprostenol (Flolan®) or alprostadil) is appropriate.
Infected ulcers (suggested by swelling, excessive pain, drainage, or discoloration of the digit) need
local wound care and a prolonged course of appropriate antibiotics. Please contact your physician for
further evaluation if infection is suspected.