Raynaud's Phenomenon Overview from Rheumatology
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:
- Rheumatic diseases - Scleroderma, systemic lupus erythematosus, mixed connective tissue disease, Sjögren’s syndrome, dermatomyositis, etc.
- Vibrating tools - Jackhammer, power drill, etc (vibration white finger)
- Drugs - Sympathomimetics (antihistamines, ephedrine, epinephrine, phenylpropanolamine)
Migraine remedies (ergot alkaloids, triptans)
Some chemotherapeutic agents (bleomycin)
- Chemicals - Nicotine, cocaine, vinyl chloride (occupational exposure)
- Occlusive vascular disease - Peripheral vascular disease, Buerger’s disease, thoracic outlet syndrome
- Hyperviscosity - Cryoglobulinemia, paraproteinemias
- 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.
If medical therapy fails, surgical procedures like sympathectomy often provide temporary relief, which could be considered along with continued drug therapy.