Definition
According to the U.S. National Heart, Lung, and Blood Institute, Raynaud's phenomenon is characterized by episodic vasospastic attacks resulting in digital ischemia. Fingers are most often affected, rarely toes. Sudden spasmodic contractions of arterioles occur when patient is exposed to cold or becomes stressed; episodes are intermittent and may last minutes or hours. Approximately 5% to 10% of the United States population is affected, with women being affected five times more often than men. It occurs usually between the ages of 20 to 40 in women and later in life for men. An estimated 80% to 90% of women with scleroderma have Raynaud's phenomenon. The two major subtypes are:
* Raynaud's disease or syndrome (primary/idiopathic), in which cases persist for more than two to three years without evidence of an associated disease. Progressive disease spasms become more frequent and more severe.
* Secondary Raynaud's phenomenon, a less common form resulting from underlying disease, such as connective tissue disease. Increased morbidity and poorer prognosis.
Etiology
Biologic factors (e.g., abnormalities in the adrenergic receptor); psychodynamic influences (e.g., stress); underlying disease (e.g., systemic lupus erythematosus)
Risk Factors
* Cigarette smoking
* Age in women (onset primarily between the ages of 20 and 40)
* Occupation (using vibrating tools like chainsaws and jackhammers)
* Pharmaceuticals (including ergot preparations) methysergide, beta-adrenergic receptor antagonists, chemotherapeutic agents such as bleomycin, vinblastine, cisplatin; and some over-the-counter cold medications and prescription narcotics
* Existing autoimmune or connective tissue disorder
* Electric shock injury
* Previous frostbite
* Repeated physical stress (such as that resulting from typing or piano playing)
* Primary pulmonary hypertension
* Exposure to cold
Signs and Symptoms
* Changes in skin color in the fingers or toes and sometimes in the nose, legs, or earlobes (may occur in three phases: pallor, cyanosis, rubor)
* Throbbing, tingling, numbness, and pain
* Atrophy of the terminal fat pads
* Gangrenous ulcers near fingertips
Differential Diagnosis
* Rheumatoid arthritis
* Systemic sclerosis (including more localized CREST)
* Systemic lupus erythematosus
* Mixed connective tissue disease
* Thromboangiitis
* Thoracic outlet compression syndrome
* Carpal tunnel syndrome
* Acrocyanosis
* Cryoglobulinemia
* Reflex sympathetic dystrophy
Diagnosis
Physical Examination
White-blue-red sequence of changes in digits after exposure to cold or emotional disturbance; secondary form may present pitting scars and ulcers of the skin or gangrene in fingers or toes.
Laboratory Tests
A variety of laboratory tests may reveal abnormalities, but they are not considered diagnostic alone. These include:
* Nail fold capillaroscopy, to distinguish between primary and secondary forms
* Antinuclear antibody test (ANA), to assess for antibodies due to connective tissue disease or other autoimmune disorder
* Erythrocyte sedimentation rate (ESR), to measure inflammation
Pathology/Pathophysiology
Appears to result from an exaggeration of normal physiological responses involving vasculature constriction. May include episodic digital ischemia secondary to exaggerated reflex sympathetic vasoconstriction; enhanced digital vascular responsiveness to cold or normal sympathetic stimuli; normal reflex sympathetic vasoconstriction superimposed on local digital vascular disease; enhanced adrenergic neuroeffector activity.
Other Diagnostic Procedures
* Clinician interview—assess symptoms and degree of severity; detailed medical history and patient report essential since attacks are intermittent; if psychological in origin, patient may have an attack due to stress when being examined
* Evaluate for medical conditions, stress, possible pharmacological side effects
* The Allen test (radial/ulnar arteries) may help distinguish between occlusion vs. vasospasm. The Taylor-Pelmear scale system is used to classify vibration-induced disease. Provocative exposure to cold.
Treatment Options
Treatment Strategy
The primary goal is to reduce frequency and severity of episodes, prevent tissue damage, and treat underlying disease if present. Conservative nondrug and self-help measures (e.g., dressing warmly and avoiding the cold, smoking cessation) are used for mild and infrequent episodes. For severe cases, options include:
* Pharmacotherapy
* Sympathectomy (if symptoms are progressive)
Drug Therapies
* Calcium-channel blockers (vasodilators) such as nifedipine (sustained-release, 10 to 30 mg tid) and diltiazem (30 to 90 mg tid) may benefit severe cases, especially with presence of peripheral vasoconstriction without significant organic vascular disease; side effects include headache, dizziness, flushing, palpitations, dyspepsia, pruritus, and edema.
* Adrenergic blocking agents such as resperine (0.25 to 0.5 mg tid) may increase nutritional blood flow to digits; side effects include hypotension, nasal stuffiness, lethargy, and depression.
* Postsynaptic alpha-adrenergic antagonist such as prazosin (1 to 5 mg tid)
* Doxazosin and terazosin
* Sympatholytic agents, including methyldopa, guanethidine, and phenoxybenzamine
* Prostaglandins to inhibit platelet aggregation; side effects include voiding and/or diarrhea and hypertension
* Angiotensin-converting enzyme inhibitors such as captopril reduce peripheral resistance, sympathetic nervous system activity, and norepinephrine release due to angiotensin suppression.
* Serotonin S2 antagonist (Ketanserin) antagonizes serotonin S2 and blocks adrenergic receptors to inhibit vasoconstriction and platelet aggregation stimulated by serotonin; not available in the United States.
* Nitroglycerine relaxes smooth muscle, dilates veins, lowers oxygen need in the myocardial tissues, inhibits platelet aggregation.
Complementary and Alternative Therapies
Raynaud's is a poorly understood syndrome that may be helped with alternative therapies that improve circulation and support general health. Begin with nutritional support and circulatory stimulants.
Biofeedback may allow patient to bring digit temperature under voluntary control. Autogenic training has been found to be an effective technique for Raynaud's phenomenon.
Nutrition
* Vitamin E (400 to 800 IU/day) improves circulation and inhibits platelet aggregation.
* Vitamin C (1,000 mg bid to tid) supports connective tissue and reduces inflammation.
* B-complex (50 to 100 mg/day) reduces the effects of stress.
* Coenzyme Q10 (100 mg bid) enhances tissue oxygenation.
* Calcium (1,500 mg/day) and magnesium (200 mg tid) relieve spasm.
* Omega-3 oils (1,500 mg bid to tid) are anti-inflammatory and inhibit platelet aggregation.
* Zinc (30 to 50 mg/day) is required for normal immune function.
Herbs
Herbs are generally a safe way to strengthen and tone the body's systems. As with any therapy, it is important to ascertain a diagnosis before pursuing treatment. Herbs may be used as dried extracts (capsules, powders, teas), glycerites, or tinctures (alcohol extracts). Unless otherwise indicated, teas should be made with 1 tsp. herb per cup of hot water. Steep covered 10 to 20 minutes and drink 2 to 4 cups/day. Tinctures may be used singly or in combination as noted.
The following herbs are circulatory stimulants with other properties as well. Use one or more tinctures in combination, 20 to 30 drops bid.
* Hawthorn berries (Crataegus laevigata) enhance vascular integrity, and has mild vasodilatory effects
* Ginkgo (Ginkgo biloba) 120 to 160 mg/day for dried extracts, inhibits platelet aggregation
* Rosemary (Rosmarinus officinalis) is a gentle relaxant
* Ginger root (Zingiber officinale) is a mild anodyne
* Prickly ash bark (Xanthoxylum clava-herculis) enhances lymph activity and vascular integrity
Homeopathy
An experienced homeopath should assess individual constitutional types and severity of disease to select the correct remedy and potency.
Acupuncture
Acupuncture may be useful as an adjunctive therapy to increase circulation and reduce vasospasm.
Massage
Therapeutic massage or bath using essential oils may help ease symptoms. A basic formula includes six drops of a blend of nutmeg (Myristica fragrans, 15 drops), lavender (Lavandula angustifolia, 5 drops), and geranium (Pelagronium graveolens, 10 drops) for bath; add 2 tbsp. vegetable oil for massage; other essential oils used in massage include cypress (Cupressus sempervirens), neroli (Citrus aurantium), lemon (Citrus limon), or rose (Rosa gallica) in 2 tsp. base oil.
Patient Monitoring
All patients should avoid or quit smoking since nicotine is a vasoconstrictor.
Other Considerations
Prevention
Patient should protect himself or herself from exposure to cold and guard against cuts and other injury to affected areas. Exercise, such as raising arms above head and whirling them vigorously, may help circulation. Biofeedback may help patient prevent or stop attacks.
Complications/Sequelae
Dry gangrene is a serious but rare complication. Other complications include ulceration of affected parts and deformities of fingers/fingernails or toes/toenails.
Prognosis
Progressive course, yet management techniques are successful in 40% to 60% of symptoms of Raynaud's phenomenon patients. Mild cases of vibration-induced disease should recover if causal activity is avoided.
Pregnancy
Some medications used to treat Raynaud's phenomenon may affect the growing fetus.
References
Balch JF, Balch PA. Prescription for Nutritional Healing. 2nd ed. Garden City Park, NY: Avery Publishing Group; 1997.
Batchelder HJ. Allopathic specific condition review: Raynaud's disease. Protocol J Botan Med. 1996;2:134-137.
Fauci AS, Braunwald E, Isselbacher KJ, et al., eds. Harrison's Principles of Internal Medicine. 14th ed. New York, NY: McGraw-Hill; 1998.
Mitchell W, Batchelder HJ. Naturopathic specific condition review: Raynaud's disease. Protocol J Botan Med. 1996;2:138-140.
Tierney LM Jr, McPhee SJ, Papadakis MA, eds. Current Medical Diagnosis & Treatment 1999. 38th ed. Stamford, Conn: Appleton & Lange; 1999.







No comments:
Post a Comment