II. Epidemiology
- Affects 3-4% of U.S. adult population
- Predominately affects women by 3 to 1 ratio
- Onset from Puberty to age 30 years
- Family History responsible in 20-30% of patients
III. Pathophysiology
- Exaggerated response to cold Temperatures
- Increased Alpha Adrenergic Receptor responsiveness
- May be primary idiopathic or secondary cause
IV. Causes: Secondary Causes of Raynaud's Phenomenon
-
Connective Tissue Disease
- Scleroderma (95% have Raynaud's)
- Systemic Lupus Erythematosus
- Sjogren's Syndrome
- Dermatomyositis
-
Trauma
- Occupational tool use (vibratory tool)
- Carpal Tunnel Syndrome
- Occlusive vascular disease
- Atherosclerosis
- Systemic Vasculitis
- Thromboembolism
- Thromboangiitis Obliterans (Buerger's Disease)
- Medications
- See provocative factors below
- Hyperviscosity state (e.g. Polycythemia Vera)
- Paraproteinemia
- Cryoglobulinemia
V. Causes: Provocative Factors
- Tobacco
- Caffeine
- Amphetamines
- Cocaine
- Pseudoephedrine
- Phenylpropanolamine
- Ephedrine
- Phenylephrine
- Ergotamines
- Triptans
- Phentermine (Qsymia)
- Unopposed Estrogen
- Nonselective Beta Blockers
- Clonidine
- Chemotherapeutic medications (e.g. Bleomycin)
VI. Symptoms
- Hypersensitivity to cold Temperatures
- Color changes of digits during cold or stress exposure
- "White attacks" suggest severe ischemia
- Mottling with acrocyanosis is more common and benign
- Sensation of numbness, clumsiness or "pins and needles"
- One finger may be more sensitive than the others
VII. Signs
- Pallor or Cyanosis of fingers or toes
- Thumb is not involved
VIII. Evaluation
- Distal pulses
- Bruit Auscultation
- Signs of ischemia
- Allen's Test
- Assess for Thoracic Outlet Syndrome
IX. Labs
- Complete Blood Count (CBC)
- Serum Chemistry Panel (Chem7)
- Urinalysis
- Consider Antinuclear Antibody (ANA) when indicated
X. Imaging
- Consider Arterial Doppler Ultrasound
XI. Diagnosis: Cold Challenge (not necessary)
- Immerse patient's hand in ice water
- Blanching occurs in seconds
- Next Cyanosis occurs
- Rewarm hand in warm water
- Erythema and pain may occur on rewarming
XII. Complications
- Cutaneous ulceration of involved digit
- Gangrene
XIII. Management: First Line
- Conservative Measures
- Smoking Cessation
- Avoid precipitating medications (see above)
- Limit Caffeine
- Avoid cold or reduce cold exposure
- Dress warmly in loose-fitting layers for the cold
- Wear a warm hat
- Wear mittens instead of gloves, and wear stockings
- Use hand warmers (chemical heat packets)
- Use a space heater at work
- Preheat car during winter
-
Calcium Channel Blockers (Dihydropyridines)
- Nifedipine ER (Procardia) orally daily
- Amlodipine
- Other medications
- Sildenafil (Viagra)
- Nitroglycerin Ointment (Nitro-Bid 2%)
- Apply to affected fingers or toes four times daily
- Do not use with Sildenafil (risk of Hypotension)
- Sodium Nitrate with Ascorbic Acid gel
XIV. Management: Severe Ischemia (e.g. CREST Syndrome related)
- See first-line measures above
- Consider Thromboangiitis Obliterans (Buerger's Disease)
- Alpha-adrenergic blockers
- Angiotensin Receptor Blockers
- Pentoxifylline (Trental)
XV. Management: Finger Temperature Feedback
- One Protocol
- Patient places fingertip on Temperature monitor
- Tone played louder when finger gets colder
- Patient tries to warm finger to decrease noise
- Reduces symptoms by 92%
- Second Protocol
- Patient immerses hands in warm water
- Rest of patient's body cold (e.g. outside)
- Repeat tid, every other day, for 3 weeks
- New conditioned cold response: Vasodilation
XVI. Management: Severe or ischemic digital ulcers
- Intravenous Prostaglandins
- PGI2 Analog: Iloprost (not available in U.S.)
- PGI2: Epoprostenol
- Cervical sympathectomy
- Proximal sympathectomy
- Localized microsurgical digital sympathectomy
- Local chemical sympathectomy with Lidocaine
XVII. References
- (2018) Presc Lett 25(12): 70
- Oreizi-Esfahani (1996) Consultant, p. 905-12
- Wigley (1999) Consultant p. 540-54
- Comfort-Adee (1993) Am Fam Physician, 47(4): 823-9 [PubMed]
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Related Studies
Definition (MSH) | An idiopathic vascular disorder characterized by bilateral Raynaud phenomenon, the abrupt onset of digital paleness or CYANOSIS in response to cold exposure or stress. |
Definition (CHV) | blood vessel disease that causes exaggerated responses to cold and stress with poor blood circulation |
Definition (CHV) | blood vessel disease that causes exaggerated responses to cold and stress with poor blood circulation |
Definition (CHV) | blood vessel disease that causes exaggerated responses to cold and stress with poor blood circulation |
Definition (MEDLINEPLUS) |
Raynaud's disease is a rare disorder of the blood vessels, usually in the fingers and toes. It causes the blood vessels to narrow when you are cold or feeling stressed. When this happens, blood can't get to the surface of the skin and the affected areas turn white and blue. When the blood flow returns, the skin turns red and throbs or tingles. In severe cases, loss of blood flow can cause sores or tissue death. Primary Raynaud's happens on its own. The cause is not known. There is also secondary Raynaud's, which is caused by injuries, other diseases, or certain medicines. People in colder climates are more likely to develop Raynaud's. It is also more common in women, people with a family history, and those over age 30. Treatment for Raynaud's may include drugs to keep the blood vessels open. There are also simple things you can do yourself, such as
NIH: National Heart, Lung, and Blood Institute |
Definition (NCI) | An idiopathic vascular disorder characterized by ischemic attacks in the fingers, toes, ears, or nose, associated with pain and pallor. The attacks occur during exposure to cold temperatures or stress. |
Definition (CSP) | intermittent attacks of ischemia in the fingers, toes, ears, or nose, accompanied by pain, pallor, and prickling; phenomenon applies to secondary symptoms, disease when cause is unknown. |
Concepts | Disease or Syndrome (T047) |
MSH | D011928 |
ICD10 | I73.0 |
SnomedCT | 123266007, 195295006, 22954002 |
English | RAYNAUDS DISEASE, RAYNAUD DISEASE, COLD FINGERS, HEREDITARY, RAYNAUDS DIS, RAYNAUD DIS, Raynaud's disease (diagnosis), Raynauds, Raynaud disease, Raynaud Disease [Disease/Finding], raynauds disease, raynaud's disease, raynauds, Disease;Raynauds, disease raynaud's, disease raynauds, raynaud disease, raynaud s disease, disease raynaud, raynaud, Raynauds disease, Cold Fingers, Hereditary, Raynaud's Disease, Raynaud's disease, Raynaud's disease (disorder), Raynaud, Raynauds Disease, Raynaud Disease |
Dutch | fenomeen van Raynaud, ziekte van Raynaud, Ziekte van Raynaud, Raynaud, ziekte van |
French | Raynaud, Maladie de Raynaud, Syndrome de Raynaud |
German | Raynaud, Raynaud-Erkrankung, Raynaud-Krankheit |
Italian | Raynaud, Malattia di Raynaud, Morbo di Raynaud |
Spanish | Raynaud, enfermedad de Raynaud - RETIRADO - (concepto no activo), enfermedad de Raynaud - RETIRADO -, enfermedad de Raynaud (trastorno), enfermedad de Raynaud, Enfermedad de Raynaud |
Swedish | Raynauds sjukdom |
Japanese | レイノービョウ, レイノー病, 対称性壊疽, レーノー病, Raynaud病 |
Czech | Raynaudova nemoc, Raynaudův fenomén, Raynaudova choroba |
Finnish | Raynaud'n oireyhtymä |
Russian | REINO BOLEZN', РЕЙНО БОЛЕЗНЬ |
Croatian | RAYNAUDOVA BOLEST |
Polish | Choroba Raynauda, Objaw Raynauda |
Hungarian | Raynaud, Raynaud-betegség |
Norwegian | Likfingre, Raynauds sykdom |
Portuguese | Doença de Raynaud |
Ontology: Raynaud Phenomenon (C0034735)
Definition (NCI_FDA) | Intermittent bilateral attacks of ischemia of the fingers or toes and sometimes of the ears or nose, marked by severe pallor, and often accompanied by paresthesia and pain. |
Definition (NCI) | A set of symptoms characteristic of peripheral vascular disease, namely caused by an inappropriate response of the peripheral arteries in reaction to environmental stimuli, usually to the cold. The term is used when an underlying disease (mostly connective tissue/autoimmune disorders such as lupus, scleroderma, rheumatoid arthritis, etc.) is primarily causative and a range of symptoms including the peripheral vascular spasm is secondary. The term does not refer to the primary Raynaud's or Raynaud's disease. |
Concepts | Disease or Syndrome (T047) |
MSH | D011928 |
ICD9 | 443.0 |
ICD10 | I73.0 |
SnomedCT | 266261006, 195297003, 195294005, 195296007, 155429004, 22954002, 266319002, 73483003 |
English | Raynaud's syndrome, RAYNAUD'S PHENOMENON, Raynaud's syndrome NOS, Raynauds Phenomenon, Raynaud's phenomenon (physical finding), Raynaud's phenomenon (diagnosis), Raynaud's Syndrome, Raynauds' phen, Raynaud phenomenon, raynaud syndrome, raynaud's syndrome, phenomenon raynauds, raynauds's syndrome, syndrome raynaud, phenomenon raynaud's, Raynauds phenomenon, raynauds syndrome, raynaud phen, raynaud phenomenon, raynaud's phenomenon, phen raynauds, raynauds' phen, Raynaud's syndrome (disorder), Raynaud's syndrome NOS (disorder), PHENOMENON, RAYNAUDS, RAYNAUDS PHENOMENON, Raynaud's syndrome (diagnosis), Raynaud's phenomenon, Paroxysmal digital cyanosis, cyanosis; paroxysmal digital, Raynaud; phenomenon, paroxysmal; digital cyanosis, phenomenon; Raynaud, Raynaud's syndrome (disorder) [Ambiguous], Raynaud Phenomenon, Raynaud's phenomenon, secondary, raynauds phenomenon, Raynaud's phenomenon (disorder), Raynaud's phenomenon (finding) |
Spanish | fenómeno de Raynaud (hallazgo), Síndrome de Raynaud, síndrome de Raynaud, SAI, síndrome de Raynaud, síndrome de Raynaud, SAI (trastorno), síndrome de Raynaud (trastorno), fenómeno de Raynaud (trastorno), cianosis digital paroxística, fenómeno de Raynaud (concepto no activo), fenómeno de Raynaud, síndrome de Raynaud (concepto no activo), Fenómeno de Raynaud |
Italian | Fenomeno di Raynaud, Sindrome di Raynaud |
Dutch | syndroom van Raynaud, Raynaud; fenomeen, cyanose; paroxysmaal digitaal, fenomeen; Raynaud, paroxysmaal; digitale cyanose, Syndroom van Raynaud, fenomeen van Raynaud |
French | Phén de Raynaud, Phénomène de Raynaud, PHENOMENE DE RAYNAUD, Syndrome de Raynaud |
German | Raynaud-Phaen, Raynaud-Syndrom, RAYNAUD PHAENOMEN, Raynaud Syndrom |
Portuguese | Síndrome de Raynaud, FENOMENO DE RAYNAUD, Fenómeno de Raynaud |
Japanese | レイノー現象, レイノー症候群, レイノーショウコウグン, レイノーゲンショウ |
Czech | Raynaudův syndrom, Raynaudův fenomén |
Korean | 레이노 증후군 |
Hungarian | Raynau-phenomen, Raynaud-syndroma, Raynaud-phenomen |
Norwegian | Raynauds fenomen |