Raynaud's phenomenon

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Raynaud's phenomenon
Classification and external resources
Hands with Raynaud's phenomenon
ICD-10	I73.0
ICD-9	443.0
DiseasesDB	25933
eMedicine	med/1993
MeSH	D011928

In medicine, Raynaud's phenomenon (pronounced /reɪˈnoʊz/, us dict: rā·nōz′) is a vasospastic disorder causing discoloration of the fingers, toes, and occasionally other extremities. This condition can also cause nails to become brittle with longitudinal ridges. Named for French physician Maurice Raynaud (1834–1881), the cause of the phenomenon is believed to be the result of vasospasms that decrease blood supply to the respective regions. Emotional stress and cold are classic triggers of the phenomenon, and the discoloration follows a characteristic pattern in time: white, blue and red.

It comprises both Raynaud's disease (primary Raynaud's), where the phenomenon is idiopathic,^[1] and Raynaud's syndrome (secondary Raynaud's), where it is caused by some other instigating factor. Measurement of hand-temperature gradients is one tool used to distinguish between the primary and secondary forms.^[2]

It is possible for the primary form to progress to the secondary form.^[3]

Prevalence

The phenomenon is more common in women than men, with the Framingham Study finding that 5% of men and 8% of women suffer from it.^{[verification needed]}

Epidemiology

There is a familial component to primary Raynaud's, and presentation is typically before two. Smoking worsens frequency and intensity of attacks, and there is a hormonal component. Caffeine also worsens the attacks. Sufferers are more likely to have migraine and angina than controls.

Secondary Raynaud's has a number of associations:

• Connective tissue disorders:
  • scleroderma^[4]
  • systemic lupus erythematosus
  • rheumatoid arthritis
  • Sjögren's syndrome
  • dermatomyositis
  • polymyositis
  • mixed connective tissue disease
  • cold agglutinin disease
• Eating disorders
  • anorexia nervosa
• Obstructive disorders
  • atherosclerosis
  • Buerger's disease
  • Takayasu's arteritis
  • subclavian aneurysms
  • thoracic outlet syndrome
• Drugs
  • Beta-blockers
  • cytotoxic drugs - particularly chemotherapeutics and most especially bleomycin
  • ciclosporin
  • ergotamine
  • sulfasalazine
  • anthrax vaccines whose primary ingredient is the Anthrax Protective Antigen
• Occupation
  • jobs involving vibration, particularly drilling
  • exposure to vinyl chloride, mercury
  • exposure to the cold (e.g. by working packing frozen food)
• Others
  • hypothyroidism
  • cryoglobulinemia
  • malignancy
  • reflex sympathetic dystrophy
  • Magnesium Deficiency
  • Erythromelalgia

It is important to realise that Raynaud's can herald these diseases by periods of more than 20 years in some cases, making it effectively their first presenting symptom. This can be the case in the CREST syndrome, of which Raynaud's is a part.

Symptoms

The condition causes painful, pale, cold extremities. This can often be distressing to those who are not diagnosed, and sometimes it can be obstructive. If someone with Raynaud's is placed in too cold a climate, it could potentially become dangerous.

Unilateral Raynaud's, or that which is present only in the hands or feet, is almost certainly primary, and will probably not progress to a secondary condition. In pregnancy, this sign normally disappears due to increased surface blood flow. Raynaud's has also occurred in breastfeeding mothers, causing nipples to turn white and become extremely painful.^[5] Nifedipine, a calcium channel blocker and vasodilator was recommended to increase blood flow to the extremities and noticeably relieved pain to the breast, in an extremely small study group.^[6]

Examination

A careful history will often reveal whether the condition is primary or secondary. Once this has been established, an examination is largely to identify or exclude possible secondary causes.

• Digital artery pressure: pressures are measured in the arteries of the fingers before and after the hands have been cooled. A decrease of at least 15mmHg is diagnostic (positive).
• Doppler ultrasound: to assess flow
• Full blood count: this can reveal a normocytic anaemia suggesting the anaemia of chronic disease or renal failure
• Urea & Electrolytes: this can reveal renal impairment
• Thyroid function tests: this can reveal hypothyroidism
• An autoantibody screen, tests for rheumatoid factor, Erythrocyte sedimentation rate and C-reactive protein, which may reveal specific causative illnesses or a generalised inflammatory process
• Nail fold vasculature: this can be examined under the microscope

Pathophysiology

Primary Raynaud phenomenon, stemming from Raynaud disease, is an exaggeration of vasomotor responses to cold or emotional stress. More specifically, it is a hyperactivation of the sympathetic system causing extreme vasoconstriction of the peripheral blood vessels, leading to tissue hypoxia. Chronic, recurrent cases of Raynaud phenomenon can result in atrophy of the skin, subcutaneous tissues, and muscle. It can also rarely cause ulceration and ischemic gangrene.^[7] Erythromelalgia is the opposite of Raynaud's where the ends turn red hot from heat rather than cold.^[8]

Treatment

Treatment options are dependent on the type of Raynaud's present. Raynaud's syndrome is treated primarily by addressing the underlying cause, but includes all options for Raynaud's disease as well. Treatment of primary Raynaud's focuses on avoiding triggers:

General Care

• Avoid environmental triggers, e.g. cold, vibration, etc. Emotional stress is another recognized trigger; although the various sources of stress can not all be avoided, it is possible to learn healthier, more effective ways of dealing with them, which will reduce stress and its somatic pathology (damaging physical effects) overall.
• Keep your hands, feet and head warm—especially your fingers, toes, ears and nose—by wearing mittens, insulated footwear, a ski mask; by using hand- and foot warmers (such as HeatBands), etc.
• Quit smoking.
• Avoid caffeine and other stimulants and vasoconstrictors that have not been prescribed to you by your doctor. Read product labels; caffeine is found not only in coffee and tea, stay-awake pills, many soft drinks and candies, but also in some cosmetics, soaps and shampoos, and even the fabric of undergarments.
• Make sure all your doctors know about all the medicines you take and about all the OTC remedies you use, especially hormones and drugs that regulate hormones, such as hormonal contraception, so that these professionals can make an assessment of your chemical regimen and make any changes that may be indicated. Contraception which is low in estrogen is preferable, and the progesterone only pill is often prescribed for women with Raynaud's.
• If you are diabetic, follow your diabetes treatment plan.

Emergency Measures

• If white finger (Raynaud's) occurs unexpectedly and a source of warm water is available, allow tepid to slightly warm water to run over the affected digits while you gently massage the area. Continue this process until the white area returns to its normal, healthy color.
• If triggered by exposure in a cold environment, and no warm water is available, place the affected digits in a warm body cavity - arm pit, crotch, or even in the mouth. Keep the affected area warm at least until the whiteness returns to its normal, healthy color. Get out of the cold as soon as possible.

Drug Therapy

• Drug treatment is normally with a calcium channel blocker, frequently nifedipine to prevent arterioconstriction.^[9][10] It has the usual common side effects of headache, flushing, and ankle edema; but these are not typically of sufficient severity to require cessation of treatment.^[11]
• There is some evidence that Angiotensin II receptor antagonists (often Losartan) reduce frequency and severity of attacks,^[12] and possibly better than nifedipine.^[13]
• Alpha-1 adrenergic blockers such as prazosin can be used to control Raynaud's vasospasms under supervision of a health care provider.^[14]
• In a study published in the November 8, 2005 issue of Circulation, sildenafil (Viagra) improved both microcirculation and symptoms in patients with secondary Raynaud's phenomenon resistant to vasodilatory therapy. The authors, led by Dr Roland Fries (Gotthard-Schettler-Klinik, Bad Schönborn, Germany), report: "In the present study, capillary blood flow was severely impaired and sometimes hardly detectable in patients with Raynaud's phenomenon. Sildenafil led to a more than 400% increase of flow velocity."^[15]

Surgical Intervention

• In intractable cases, sympathectomy^[16] and infusions of prostaglandins, e.g. prostacyclin, may be tried, with amputation in exceptionally severe cases.

Alternative and Experimental (Research) Approaches

• The extract of the Ginkgo biloba leaves (Egb 761, 80 mg) may reduce frequency of attacks.^[17]
• Two separate gels combined on the fingertip (somewhat like two-part epoxy, they cannot be combined before use because they will react) increased blood flow in the fingertips by about three times. One gel contained 5% sodium nitrite and the other contained 5% ascorbic acid. The milliliter of combined gel covered an area of ~3 cm². The gel was wiped off after a few seconds.^[18]
• Piracetam

See also

• CREST syndrome
• Circulatory system

References

External links

• Overview at National Institutes of Health
• Overview at Medical College of Wisconsin

• Raynaud's Phenomenon at YouTube (requires Adobe Flash)

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