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In [[medicine]], '''Raynaud's phenomenon''' ({{IPAc-en|icon|r|eɪ|ˈ|n|oʊ|z}}) is a [[vasospasm|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.
In [[medicine]], '''Raynaud's phenomenon''' ({{IPAc-en|Raynauds sucks. icon|r|eɪ|ˈ|n|oʊ|z}}) is a [[vasospasm|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]],<ref>{{DorlandsDict|nine/000956360|Raynaud disease}}</ref> 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.<ref name="pmid17018538">{{cite journal |author=Anderson ME, Moore TL, Lunt M, Herrick AL |title=The 'distal-dorsal difference': a thermographic parameter by which to differentiate between primary and secondary Raynaud's phenomenon |journal=Rheumatology |volume=46 |issue=3 |pages=533–8 |year=2007 |month=March |pmid=17018538 |doi=10.1093/rheumatology/kel330}}</ref>
It comprises both '''Raynaud's disease''' (also known as "Primary Raynaud's phenomenon") where the phenomenon is [[idiopathic]],<ref>{{DorlandsDict|nine/000956360|Raynaud disease}}</ref> 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.<ref name="pmid17018538">{{cite journal |author=Anderson ME, Moore TL, Lunt M, Herrick AL |title=The 'distal-dorsal difference': a thermographic parameter by which to differentiate between primary and secondary Raynaud's phenomenon |journal=Rheumatology |volume=46 |issue=3 |pages=533–8 |year=2007 |month=March |pmid=17018538 |doi=10.1093/rheumatology/kel330}}</ref>

Revision as of 19:11, 16 September 2011

Raynaud syndrome
SpecialtyImmunology, rheumatology Edit this on Wikidata

In medicine, Raynaud's phenomenon (/[invalid input: 'Raynauds sucks. icon']reɪˈnoʊz/) 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]

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, tingling, and a painful "pins and needles" sensation.

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 Raynaud Phenomenon.

Raynaud phenomenon results from an exaggerated vasoconstriction of digital arteries and arterioles. These vascular changes induce paroxysmal pallor or cyanosis of the digits of the hands or feet; infrequently, the nose, earlobes, or lips can also be involved. Characteristically, the involved digits show red, white, and blue color changes from most proximal to most distal, correlating with proximal vasodilation, central vasoconstriction, and more distal cyanosis ( Fig. 11-28 ). Raynaud phenomenon may be a primary disease entity or be secondary to a variety of conditions.[82]

FIGURE 11-28 Raynaud's phenomenon. A, Sharply demarcated pallor of the distal fingers resulting from the closure of digital arteries. B, Cyanosis of the fingertips. (Reproduced from Salvarani C, et al.: Polymyalgia rheumatica and giant-cell arteritis. N Engl J Med 347:261, 2002.)

Primary Raynaud phenomenon (previously called Raynaud disease) reflects an exaggeration of central and local vasomotor responses to cold or emotional stresses. It affects 3% to 5% of the general population and shows a predilection for young women. Structural changes in the arterial walls are absent except late in the course, when intimal thickening can appear. The course of Raynaud phenomenon is usually benign, but when long-standing can result in atrophy of the skin, subcutaneous tissues, and muscles. Ulceration and ischemic gangrene are rare.[83]

In contrast, secondary Raynaud phenomenon refers to vascular insufficiency of the extremities secondary to arterial disease caused by other entities including SLE, scleroderma, Buerger disease, or even atherosclerosis. Since Raynaud phenomenon may be the first manifestation of such conditions, any patient with new symptoms should be evaluated. Of these individuals, some 10% will eventually manifest an underlying disease.[verification needed]

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, if 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:

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.

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.
  • Keep your hands, feet and head warm—especially your fingers, toes, ears, and nose — by wearing mittens, insulated footwear, a ski mask; or using hand and foot warmers, etc.
  • Quit smoking.
  • Avoid caffeine and other stimulants and vasoconstrictors. Caffeine is found not only in coffee and tea, stay-awake pills, soft drinks, and candies, but also in some cosmetics, soaps, and shampoos(shower shock caffeinated bar and alpecin shampoo).
  • Hormones and drugs that regulate hormones, such as hormonal contraception may aggravate Raynaud's. Contraception which is low in estrogen is preferable, and the progesterone only pill is often prescribed for women with Raynaud's.
  • Calcium channel blockers can be helpful for the treatment of Raynaud's phenomenon.

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.
  • A useful method exists to take measures ensuring circulation is restored quickly, though this should not be attempted if other circulatory issues are present that disallow fast or vigorous movement. This method is achieved by "windmilling" the arms (swinging them in large circles) reasonably fast to force the blood to the hands and fingers. This can cause dizziness, so it should be attempted only for a short time before stopping for a minute and subsequently continuing. A less effective (but less conspicuous) variation is to swing the arms back and forth in a part of a circle to achieve the same results. This variation does take longer, though, and requires swifter movement of the arms, so it is advised to use the first variation if 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.
  • RayVa is a topical cream for the treatment of Raynaud's phenomenon. It contains prostaglandin E1 as the active ingredient and a permeation enhancer DDAIP which facilitates the delivery of the drug into the blood stream.

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 Ayurvedic oil preparation Mahanarayan Oil has been used very successfully in many cases as a vasodilater when applied daily to affected areas. [citation needed]
  • 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.[citation needed]
  • Arginine, which increase nitric oxide acts as a vasodilator.[citation needed]
  • 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

Footnotes

  1. ^ "Raynaud disease" at Dorland's Medical Dictionary
  2. ^ Anderson ME, Moore TL, Lunt M, Herrick AL (2007). "The 'distal-dorsal difference': a thermographic parameter by which to differentiate between primary and secondary Raynaud's phenomenon". Rheumatology. 46 (3): 533–8. doi:10.1093/rheumatology/kel330. PMID 17018538. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  3. ^ Hirschl M, Hirschl K, Lenz M, Katzenschlager R, Hutter HP, Kundi M (2006). "Transition from primary Raynaud's phenomenon to secondary Raynaud's phenomenon identified by diagnosis of an associated disease: results of ten years of prospective surveillance". Arthritis and Rheumatism. 54 (6): 1974–81. doi:10.1002/art.21912. PMID 16732585. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  4. ^ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease. St. Louis, Mo: Elsevier Saunders. p. 542. ISBN 0-7216-0187-1.{{cite book}}: CS1 maint: multiple names: authors list (link)
  5. ^ Holmen OL, Backe B (2009). "An underdiagnosed cause of nipple pain presented on a camera phone". BMJ. 339: b2553. doi:10.1136/bmj.b2553.
  6. ^ Anderson JE, Held N, Wright K (2004). "Raynaud's phenomenon of the nipple: a treatable cause of painful breastfeeding". Pediatrics. 113 (4): e360–4. doi:10.1542/peds.113.4.e360. PMID 15060268. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  7. ^ Pistorius MA, Planchon B, Schott JJ, Lemarec H (2006). "[Heredity and genetic aspects of Raynaud's disease]". Journal Des Maladies Vasculaires (in French). 31 (1): 10–5. PMID 16609626. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  8. ^ Gayraud M (2007). "Raynaud's phenomenon". Joint, Bone, Spine. 74 (1): e1–8. doi:10.1016/j.jbspin.2006.07.002. PMID 17218139. {{cite journal}}: Unknown parameter |month= ignored (help)
  9. ^ Berlin AL, Pehr K (2004). "Coexistence of erythromelalgia and Raynaud's phenomenon". Journal of the American Academy of Dermatology. 50 (3): 456–60. doi:10.1016/S0190-9622(03)02121-2. PMID 14988692. {{cite journal}}: Unknown parameter |month= ignored (help)
  10. ^ Priollet P (1998). "[Raynaud's phenomena: diagnostic and treatment study]". La Revue Du Praticien (in French). 48 (15): 1659–64. PMID 9814067. {{cite journal}}: Unknown parameter |month= ignored (help)
  11. ^ Kahan A, Weber S, Amor B, Saporta L, Hodara M, Degeorges M (1981). "Nifedipine and Raynaud's phenomenon". Annals of Internal Medicine. 94 (4 pt 1): 546. PMID 7212523. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  12. ^ Kahan A, Weber S, Amor B, Saporta L, Hodara M, Degeorges M (1982). "[Controlled study of nifedipine in the treatment of Raynaud's phenomenon]". Revue Du Rhumatisme et Des Maladies Ostéo-articulaires (in French). 49 (5): 337–43. PMID 6285445. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  13. ^ Smith CR, Rodeheffer RJ (1985). "Raynaud's phenomenon: pathophysiologic features and treatment with calcium-channel blockers". The American Journal of Cardiology. 55 (3): 154B–157B. doi:10.1016/0002-9149(85)90625-3. PMID 3881908. {{cite journal}}: Unknown parameter |month= ignored (help)
  14. ^ Pancera P, Sansone S, Secchi S, Covi G, Lechi A (1997). "The effects of thromboxane A2 inhibition (picotamide) and angiotensin II receptor blockade (losartan) in primary Raynaud's phenomenon". Journal of Internal Medicine. 242 (5): 373–6. doi:10.1046/j.1365-2796.1997.00219.x. PMID 9408065. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  15. ^ Dziadzio M, Denton CP, Smith R; et al. (1999). "Losartan therapy for Raynaud's phenomenon and scleroderma: clinical and biochemical findings in a fifteen-week, randomized, parallel-group, controlled trial". Arthritis and Rheumatism. 42 (12): 2646–55. doi:10.1002/1529-0131(199912)42:12<2646::AID-ANR21>3.0.CO;2-T. PMID 10616013. {{cite journal}}: Explicit use of et al. in: |author= (help); Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  16. ^ Waldo R (1979). "Prazosin relieves Raynaud's vasospasm". JAMA. 241 (10): 1037. doi:10.1001/jama.241.10.1037. PMID 762741. {{cite journal}}: Unknown parameter |month= ignored (help)
  17. ^ Fries R, Shariat K, von Wilmowsky H, Böhm M (2005). "Sildenafil in the treatment of Raynaud's phenomenon resistant to vasodilatory therapy". Circulation. 112 (19): 2980–5. doi:10.1161/CIRCULATIONAHA.104.523324. PMID 16275885. {{cite journal}}: Unknown parameter |doi_brokendate= ignored (|doi-broken-date= suggested) (help); Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  18. ^ Wang WH, Lai CS, Chang KP; et al. (2006). "Peripheral sympathectomy for Raynaud's phenomenon: a salvage procedure". The Kaohsiung Journal of Medical Sciences. 22 (10): 491–9. doi:10.1016/S1607-551X(09)70343-2. PMID 17098681. {{cite journal}}: Explicit use of et al. in: |author= (help); Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  19. ^ Neumeister MW, Chambers CB, Herron MS; et al. (2009). "Botox therapy for ischemic digits". Plastic and Reconstructive Surgery. 124 (1): 191–201. doi:10.1097/PRS.0b013e3181a80576. PMID 19568080. {{cite journal}}: Explicit use of et al. in: |author= (help); Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  20. ^ Van Beek AL, Lim PK, Gear AJ, Pritzker MR (2007). "Management of vasospastic disorders with botulinum toxin A". Plastic and Reconstructive Surgery. 119 (1): 217–26. doi:10.1097/01.prs.0000244860.00674.57. PMID 17255677. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  21. ^ Muir AH, Robb R, McLaren M, Daly F, Belch JJ (2002). "The use of Ginkgo biloba in Raynaud's disease: a double-blind placebo-controlled trial". Vascular Medicine. 7 (4): 265–7. doi:10.1191/1358863x02vm455oa. PMID 12710841.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  22. ^ Tucker AT, Pearson RM, Cooke ED, Benjamin N (1999). "Effect of nitric-oxide-generating system on microcirculatory blood flow in skin of patients with severe Raynaud's syndrome: a randomised trial". Lancet. 354 (9191): 1670–5. doi:10.1016/S0140-6736(99)04095-7. PMID 10568568. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  23. ^ Karavidas MK, Tsai PS, Yucha C, McGrady A, Lehrer PM (2006). "Thermal biofeedback for primary Raynaud's phenomenon: a review of the literature". Applied Psychophysiology and Biofeedback. 31 (3): 203–16. doi:10.1007/s10484-006-9018-2. PMID 17016765. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  24. ^ DiGiacomo RA, Kremer JM, Shah DM (1989). "Fish-oil dietary supplementation in patients with Raynaud's phenomenon: a double-blind, controlled, prospective study". The American Journal of Medicine. 86 (2): 158–64. doi:10.1016/0002-9343(89)90261-1. PMID 2536517. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
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