Raynaud's phenomenon

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 areas. This condition can also cause nails to become brittle with longitudinal ridges. Named for French physician Maurice Raynaud (1834–1881), 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.

It comprises both Raynaud's disease (also known as "Primary Raynaud's phenomenon") 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]

In extreme cases, the secondary form can progress to necrosis or gangrene of the fingertips.

Raynaud's phenomenon 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. In rare cases it can cause ulceration and ischemic gangrene.^[4]

Contents 1 Symptoms 2 Prevalence 3 Epidemiology 3.1 Primary Raynaud's (disease) 3.2 Secondary Raynaud's (syndrome) 4 Examination 5 Treatment 5.1 General care 5.2 Emergency measures 5.3 Drug therapy 5.4 Surgical Intervention 5.5 Alternative and Experimental (Research) Approaches 6 See also 7 Footnotes 8 External links

Symptoms

The condition can cause pain within the affected extremities, discoloration (paleness) and sensations of cold and/or numbness. 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.

The symptoms include several cyclic color changes:

1. 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.
2. When the oxygen supply is depleted, the skin colour turns blue (called cyanosis).
3. These events are episodic, and when the episode subsides or the area is warmed, the blood flow returns and the skin colour first turns red (rubor), and then back to normal, often accompanied by swelling and tingling.

All three colour changes are observed in classic Raynaud's. However, not all patients see all of the aforementioned colour changes in all episodes, especially in milder cases of the condition. Symptoms are thought to be due to reactive hyperemias of the areas deprived of blood flow.

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]

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.

Epidemiology

It is important to distinguish Raynaud's disease from syndrome. In order to diagnose these two forms of Raynaud's, a doctor may look for signs of arthritis or vasculitis, and may conduct a number of laboratory tests.

Primary Raynaud's (disease)

Raynaud's disease, or "Primary Raynaud's", is diagnosed if the symptoms are idiopathic, that is, they occur by themselves and not in association with other diseases. Some refer to Primary Raynaud's disease as "being allergic to coldness". It often develops in young women in their teens and early adulthood. Primary Raynaud's is thought to be at least partly hereditary, although specific genes have not yet been identified.^[7]

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 (syndrome)

Raynaud's syndrome, or "Secondary Raynaud's", occurs secondary to a wide variety of other conditions. Secondary Raynaud's has a number of associations:

• Connective tissue disorders:
  • scleroderma^[8]
  • systemic lupus erythematosus
  • rheumatoid arthritis
  • Sjögren's syndrome
  • dermatomyositis
  • polymyositis
  • mixed connective tissue disease
  • cold agglutinin disease
  • Ehlers-Danlos Syndrome
• 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 suffer from Vibration white finger
  • exposure to vinyl chloride, mercury
  • exposure to the cold (e.g. by working packing frozen food)
• Others
  • hypothyroidism
  • cryoglobulinemia
  • malignancy
  • reflex sympathetic dystrophy
  • carpal tunnel syndrome
  • Magnesium Deficiency
  • Erythromelalgia, (the opposite of Raynaud's, with hot and warm extremities) often co-exists in patients with Raynaud's)^[9]

It is important to realize 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.

Patients with Secondary Raynaud's can also have symptoms related to their underlying diseases. Raynaud's phenomenon is the initial symptom that presents for 70% of patients with scleroderma, a skin and joint disease.

When Raynaud's phenomenon is limited to one hand or one foot, it is referred to as Unilateral Raynaud's. This is an uncommon form, and it is always secondary to local or regional vascular disease. It commonly progresses within several years to affect other limbs as the vascular disease progresses.^[10]

Examination

A careful medical 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 15 mmHg is diagnostic (positive).
• Doppler ultrasound: to assess blood flow.
• Full blood count: this can reveal a normocytic anaemia suggesting the anaemia of chronic disease or renal failure.
• Blood test for urea and 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.

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 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, 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(shower shock caffinated bar and alpecin sampoo).
• 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

• Treatment for Raynaud's phenomenon may include prescription medicines that dilate blood vessels, such as calcium channel blockers (nifedipine) or diltiazem.^[11][12] 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.^[13]
• There is some evidence that Angiotensin II receptor antagonists (often Losartan) reduce frequency and severity of attacks,^[14] and possibly better than nifedipine.^[15]
• Alpha-1 adrenergic blockers such as prazosin can be used to control Raynaud's vasospasms under supervision of a health care provider.^[16]
• 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."^[17]
• Fluoxetine, a selective serotonin reuptake inhibitor, and other antidepressant medications may reduce the frequency and severity of episodes if caused mainly by psychological stress.

Surgical Intervention

• In severe cases, a sympathectomy^[18] 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. Infusions of prostaglandins, e.g. prostacyclin, may be tried, with amputation in exceptionally severe cases.
• A more recent treatment for severe Raynaud's is the use of Botox. The 2009 article^[19] 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, 3 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^[20] describes similar improvement in a series of 11 patients. All patients had significant relief of pain.

Alternative and Experimental (Research) Approaches

• The extract of the Ginkgo biloba leaves (Egb 761, 80 mg) may reduce frequency of attacks.^[21]
• 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.^[22]
• Piracetam, a nootropic drug, can be useful as a long-term treatment for vasospastic disorders.
• Arginine, which increase nitrous oxide acts as a vasodilator
• Milder cases of Raynaud's can often be addressed by biofeedback^[23] or other techniques to help control involuntary body functions like skin temperature.
• Fish oil supplements which contain long-chain omega-3 fatty acids may help to control symptoms of primary Raynaud's. There are few studies in the medical literature dealing with this subject. However, in one 1989 controlled, double-blinded study of 32 patients,^[24] consumption of roughly 6.5 grams of long chain omega-3 fatty acids in the form of fish oil significantly increased the time to onset or entirely prevented symptoms in response to cold in patients with primary Raynaud's. Lower doses of fish oil such as may be commonly available from commercial vendors have not been studied and may not be as effective.

See also

• CREST syndrome
• Circulatory system
• Vasospasm
• Cryopedis

Footnotes

External links

• Overview at National Institutes of Health
• Overview at Medical College of Wisconsin
• Raynaud's Disease at healthatoz.com
• Raynaud's Phenomenon at YouTube
• Raynaud's Disease at YouTube
• Video at YouTube
• What Is Raynaud's Disease at National Heart, Lung, and Blood Institute
• Raynaud's Disease at Mayo Clinic
• Raynaud's & Scleroderma Association, a national charity and self help organisation, committed to supporting patients and carers who have these conditions
• Bakst R, Merola JF, Franks AG, Sanchez M (October 2008). "Raynaud's phenomenon: pathogenesis and management". Journal of the American Academy of Dermatology 59 (4): 633–53. doi:10.1016/j.jaad.2008.06.004. PMID 18656283.