Raynaud's phenomenon

Infobox_Disease
Name = Raynauds phenomenon


Caption = Hands with Raynaud's phenomenon
DiseasesDB = 25933
ICD10 = ICD10|I|73|0|i|70
ICD9 = ICD9|443.0
ICDO =
OMIM =
MedlinePlus =
eMedicineSubj = med
eMedicineTopic = 1993
MeshID = D011928

Raynaud's phenomenon (PronEng|reɪˈnoʊz) (rāy-NŌZ), in medicine, 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, [DorlandsDict|nine/000956360|Raynaud disease] 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.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 (Oxford) |volume=46 |issue=3 |pages=533–8 |year=2007 |pmid=17018538 |doi=10.1093/rheumatology/kel330 |url=http://rheumatology.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=17018538]

It is possible for the primary form to progress to the secondary form.cite journal |author=Hirschl M, Hirschl K, Lenz M, Katzenschlager R, Hutter HP, Kundi M |title=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 |journal=Arthritis Rheum. |volume=54 |issue=6 |pages=1974–81 |year=2006 |pmid=16732585 |doi=10.1002/art.21912]

Incidence

The phenomenon is more common in women than men, with the Framingham Study finding that 5.8% of men and 9.6% of women suffered from it.

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. Sufferers are more likely to have migraine and angina than controls.

Secondary Raynaud's has a number of associations:
*Connective tissue disorders:
**sclerodermacite journal |author=Gayraud M |title=Raynaud's phenomenon |journal=Joint Bone Spine |volume=74 |issue=1 |pages=e1–8 |year=2007 |pmid=17218139 |doi=10.1016/j.jbspin.2006.07.002 |url=http://linkinghub.elsevier.com/retrieve/pii/S1297-319X(06)00246-6]
**systemic lupus erythematosus
**rheumatoid arthritis
**Sjögren's syndrome
**dermatomyositis
**polymyositis
*Eating disorders
**Anorexia Nervosa
*Obstructive disorders
**atherosclerosis
**Buerger's disease
**subclavian aneurysms
**thoracic outlet syndrome
*Drugs
**Beta-blockers
**cytotoxic drugs - particularly chemotherapeutics and most especially bleomycin
**cyclosporin
**ergotamine
**sulfasalazine
*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

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.

ymptoms

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 secondary, as primary Raynaud's is a systemic condition. However, a patient's feet may be affected without him or her realizing it.

In pregnancy, this sign normally disappears due to increased surface blood flow.

Investigations

A careful history will often reveal whether the condition is primary or secondary. Once this has been established, investigations are largely to identify or exclude possible secondary causes.
*Digital artery pressure: pressures are measured in the digital arteries before and after cooling the hands. A drop of 15mmHg or more is diagnostic.
*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.cite book|title= Robbins & Cotran Pathologic Basis of Desease|last= Kumar|first= Vinay|coauthors= Nelso Fausto, Abul Abbas|year= 2004|publisher= Saunders|isbn= 0721601871|pages=542 ]

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 measures

*Avoidance of any environmental triggers, e.g. cold, vibration, etc. (although emotional stress is a recognized trigger, it tends to be impossible to consciously avoid).
*Warm clothing for the extremities such as mittens or HeatBands
*Hormone regulation and assessment of the type of hormonal contraception used, if any. Contraception which is low in estrogen is preferable, and the progesterone only pill is often prescribed.
*Smoking cessation.

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 gently massaging the area. Continue this process until the white area turns pink or a 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 pink or a healthy color, avoid continued exposure to the cold.

Drug therapy

*Drug treatment is normally with a calcium channel blocker, frequently nifedipine to prevent arterioconstriction.cite journal |author=Kahan A, Weber S, Amor B, Saporta L, Hodara M, Degeorges M |title=Nifedipine and Raynaud's phenomenon |journal=Ann. Intern. Med. |volume=94 |issue=4 pt 1 |pages=546 |year=1981 |pmid=7212523 |doi=] cite journal |author=Kahan A, Weber S, Amor B, Saporta L, Hodara M, Degeorges M |title= [Controlled study of nifedipine in the treatment of Raynaud's phenomenon] |language=French |journal=Rev Rhum Mal Osteoartic |volume=49 |issue=5 |pages=337–43 |year=1982 |pmid=6285445 |doi=] It has the usual common side effects of headache, flushing, and ankle edema; but normally result in not needing to stop the drug.cite journal |author=Smith CR, Rodeheffer RJ |title=Raynaud's phenomenon: pathophysiologic features and treatment with calcium-channel blockers |journal=Am. J. Cardiol. |volume=55 |issue=3 |pages=154B–157B |year=1985 |pmid=3881908|doi=10.1016/0002-9149(85)90625-3]
*There is some evidence that Angiotensin II receptor antagonists (often Losartan) reduce frequency and severity of attacks,cite journal |author=Pancera P, Sansone S, Secchi S, Covi G, Lechi A |title=The effects of thromboxane A2 inhibition (picotamide) and angiotensin II receptor blockade (losartan) in primary Raynaud's phenomenon |journal=J. Intern. Med. |volume=242 |issue=5 |pages=373–6 |year=1997 |pmid=9408065|doi=10.1046/j.1365-2796.1997.00219.x] and possibly better than nifedipine.cite journal |author=Dziadzio M, Denton CP, Smith R, "et al" |title=Losartan therapy for Raynaud's phenomenon and scleroderma: clinical and biochemical findings in a fifteen-week, randomized, parallel-group, controlled trial |journal=Arthritis Rheum. |volume=42 |issue=12 |pages=2646–55 |year=1999 |pmid=10616013 |doi=10.1002/1529-0131(199912)42:12<2646::AID-ANR21>3.0.CO;2-T |url=http://www3.interscience.wiley.com/cgi-bin/fulltext/78503570/PDFSTART |format=PDF]
*Alpha-1 adrenergic blockers such as prazosin can be used to control Raynaud's vasospasms under supervision of a health care provider.cite journal |author=Waldo R |title=Prazosin relieves Raynaud's vasospasm |journal=JAMA |volume=241 |issue=10 |pages=1037 |year=1979 |pmid=762741|doi=10.1001/jama.241.10.1037]
*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."cite journal |author=Fries R, Shariat K, von Wilmowsky H, Böhm M |title=Sildenafil in the treatment of Raynaud's phenomenon resistant to vasodilatory therapy |journal=Circulation |volume=112 |issue=19 |pages=2980–5 |year=2005 |pmid=16275885 |doi=10.1161/CIRCULATIONAHA.104.523324 |url=http://circ.ahajournals.org/cgi/content/full/112/19/2980 |doi_brokendate=2008-06-21]

urgical intervention

*In intractable cases, sympathectomycite journal |author=Wang WH, Lai CS, Chang KP, "et al" |title=Peripheral sympathectomy for Raynaud's phenomenon: a salvage procedure |journal=Kaohsiung J. Med. Sci. |volume=22 |issue=10 |pages=491–9 |year=2006 |pmid=17098681 |doi= |url=http://health.elsevier.com/ajws_pubmed/pubmed_switch.asp?journal_issn=1607-551X&art_pub_year=2006&%20art_pub_month=10&art_pub_vol=22&art_sp=491] and infusions of prostaglandins, e.g. prostacyclin, may be tried, with amputation in exceptionally severe cases.

Alternative and research approaches

*The extract of the Ginkgo biloba leaves (Egb 761, 80mg) may reduce frequency of attacks.cite journal |author=Muir AH, Robb R, McLaren M, Daly F, Belch JJ |title=The use of Ginkgo biloba in Raynaud's disease: a double-blind placebo-controlled trial |journal=Vasc Med |volume=7 |issue=4 |pages=265–7 |year=2002 |pmid=12710841|doi=10.1191/1358863x02vm455oa]
*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.cite journal |author=Tucker AT, Pearson RM, Cooke ED, Benjamin N |title=Effect of nitric-oxide-generating system on microcirculatory blood flow in skin of patients with severe Raynaud's syndrome: a randomised trial |journal=Lancet |volume=354 |issue=9191 |pages=1670–5 |year=1999 |month=Nov 13 |pmid=10568568|doi=10.1016/S0140-6736(99)04095-7]

ee also

*CREST syndrome
*Circulatory system

References

External links

* [http://www.niams.nih.gov/hi/topics/raynaud/ar125fs.htm Overview] at National Institutes of Health
* [http://healthlink.mcw.edu/article/926055412.html Overview] at Medical College of Wisconsin

*YouTube|nRtNY6x1LAY|Raynaud's Phenomenon