I. Epidemiology

  1. Affects 3-4% of U.S. adult population
  2. Predominately affects women by 3 to 1 ratio
  3. Onset from Puberty to age 30 years
  4. Family History responsible in 20-30% of patients

II. Pathophysiology

  1. Exaggerated response to cold Temperatures
  2. Increased Alpha Adrenergic Receptor responsiveness
  3. May be primary idiopathic or secondary cause

III. Secondary Causes of Raynaud's Phenomenon

  1. Connective tissue disease
    1. Scleroderma (95% have Raynaud's)
    2. Systemic Lupus Erythematosus
    3. Sjogren's Syndrome
    4. Dermatomyositis
  2. Trauma
    1. Occupational tool use (vibratory tool)
    2. Carpal Tunnel Syndrome
  3. Occlusive vascular disease
    1. Atherosclerosis
    2. Systemic Vasculitis
    3. Thromboembolism
  4. Medications
    1. See provocative factors below
  5. Hyperviscosity state (e.g. Polycythemia Vera)
  6. Paraproteinemia
  7. Cryoglobulinemia

IV. Provocative Factors

  1. Tobacco
  2. Caffeine
  3. Amphetamines
  4. Cocaine
  5. Pseudoephedrine
  6. Phenylpropanolamine
  7. Ephedrine
  8. Phenylephrine
  9. Ergotamines
  10. Unopposed Estrogen
  11. Nonselective Beta Blockers
  12. Clonidine
  13. Chemotherapeutic medications (e.g. Bleomycin)

V. Symptoms

  1. Hypersensitivity to cold Temperatures
  2. Color changes of digits during cold or stress exposure
    1. "White attacks" suggest severe ischemia
    2. Mottling with acrocyanosis more common and benign
  3. Sensation of numbness, clumsiness or "pins and needles"
  4. One finger may be more sensitive than the others

VI. Signs

  1. Pallor or Cyanosis of fingers or toes
  2. Thumb is not involved

VII. Evaluation

  1. Distal pulses
  2. Bruit Auscultation
  3. Signs of ischemia
  4. Allen's Test
  5. Assess for Thoracic Outlet Syndrome

VIII. Labs

  1. Complete Blood Count (CBC)
  2. Serum Chemistry Panel (Chem7)
  3. Urinalysis
  4. Consider Antinuclear Antibody (ANA) when indicated

IX. Radiology

  1. Consider Arterial Doppler Ultrasound

X. Diagnosis: Cold Challenge (not necessary)

  1. Immerse patient's hand in ice water
    1. Blanching occurs in seconds
    2. Next Cyanosis occurs
  2. Rewarm hand in warm water
    1. Erythema and pain may occur on rewarming

XI. Complications

  1. Cutaneous Ulceration of involved digit
  2. Gangrene

XII. Management: First Line

  1. Conservative Measures
    1. Smoking Cessation
    2. Avoid precipitating medications (see above)
    3. Avoid cold or reduce cold exposure
      1. Dress warmly in loose-fitting layers for the cold
      2. Wear a warm hat
      3. Wear mittens instead of gloves, and wear stockings
      4. Use hand warmers (chemical heat packets)
      5. Use a space heater at work
      6. Preheat car during winter
  2. Calcium Channel Blockers (Dihydropyridines)
    1. Nifedipine (Procardia) 15 to 60 mg PO qd
    2. Amlodipine
  3. Other medications
    1. Nitroglycerin Ointment
    2. Sodium Nitrate with Ascorbic Acid gel
      1. Tucker (1999) Lancet 354:1670-5

XIII. Management: Severe Ischemia (e.g. CREST Syndrome related)

  1. See first-line measures above
  2. Alpha-adrenergic blockers
  3. Angiotensin Receptor Blockers
  4. Pentoxifylline (Trental)

XIV. Management: Finger Temperature Feedback

  1. One Protocol
    1. Patient places fingertip on Temperature monitor
    2. Tone played louder when finger gets colder
    3. Patient tries to warm finger to decrease noise
    4. Reduces symptoms by 92%
  2. Second Protocol
    1. Patient immerses hands in warm water
    2. Rest of patient's body cold (e.g. outside)
    3. Repeat tid, every other day, for 3 weeks
    4. New conditioned cold response: Vasodilation

XV. Management: Severe or ischemic digital ulcers

  1. Intravenous Prostaglandins
    1. PGI2 Analog: Iloprost (not available in U.S.)
    2. PGI2: Epoprostenol
  2. Cervical sympathectomy
    1. Proximal sympathectomy
    2. Localized microsurgical digital sympathectomy
    3. Local chemical sympathectomy with Lidocaine

XVI. References

  1. Oreizi-Esfahani (1996) Consultant, p. 905-12
  2. Wigley (1999) Consultant p. 540-54
  3. Comfort-Adee (1993) Am Fam Physician, 47(4): 823-9

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Ontology: Raynaud Disease (C0034734)

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 (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 (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. People with this disorder have attacks that cause the blood vessels to narrow. 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. Cold weather and stress can trigger attacks. Often the cause of Raynaud's is not known. People in colder climates are more likely to develop Raynaud's than people in warmer areas.

Treatment for Raynaud's may include drugs to keep the blood vessels open. There are also simple things you can do yourself, such as

  • Soaking hands in warm water at the first sign of an attack
  • Keeping your hands and feet warm in cold weather

NIH: National Heart, Lung, and Blood Institute

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
ICD9 443.0
ICD10 I73.0
SnomedCT 123266007, 22954002, 195294005, 195297003, 195295006, 155429004, 266319002
English Raynaud's syndrome, RAYNAUDS DISEASE, RAYNAUDS SYNDROME, Raynaud's syndrome NOS, RAYNAUD DISEASE, COLD FINGERS, HEREDITARY, RAYNAUDS DIS, RAYNAUD DIS, Raynaud's syndrome (disorder), Raynaud's disease (diagnosis), raynaud's syndrome, Raynauds, Raynaud's syndrome NOS (disorder), Raynaud disease, Raynaud Disease [Disease/Finding], raynaud syndrome, raynauds disease, raynaud's disease, raynauds, raynauds's syndrome, syndrome raynaud, Disease;Raynauds, disease raynaud's, disease raynauds, raynaud disease, raynaud s disease, raynauds syndrome, disease raynaud, raynaud, Raynaud's Disease, Raynaud's disease, Raynaud's disease (disorder), Raynaud, Raynaud's syndrome (disorder) [Ambiguous], Raynauds Disease, Raynaud Disease, Raynauds disease
Dutch fenomeen van Raynaud, ziekte van Raynaud, syndroom van Raynaud, Syndroom van Raynaud, Ziekte van Raynaud, Raynaud, ziekte van
French Raynaud, Maladie de Raynaud, Syndrome de Raynaud
German Raynaud, Raynaud-Syndrom, Raynaud-Erkrankung, Raynaud-Krankheit
Italian Raynaud, Sindrome di Raynaud, Malattia di Raynaud, Morbo di Raynaud
Portuguese Síndrome de Raynaud, Doença de Raynaud
Spanish Síndrome de Raynaud, Raynaud, Raynaud's syndrome, enfermedad de Raynaud (trastorno), enfermedad de Raynaud - RETIRADO - (concepto no activo), enfermedad de Raynaud - RETIRADO -, enfermedad de Raynaud, síndrome de Raynaud (concepto no activo), síndrome de Raynaud (trastorno), síndrome de Raynaud, SAI (trastorno), síndrome de Raynaud, SAI, síndrome de Raynaud, Enfermedad de Raynaud
Japanese レイノー症候群, レイノーショウコウグン, レイノービョウ, レイノー病, 対称性壊疽, レーノー病, Raynaud病
Swedish Raynauds sjukdom
Czech Raynaudova nemoc, Raynaudův syndrom, Raynaudův fenomén
Finnish Raynaud'n oireyhtymä
Russian REINO BOLEZN', РЕЙНО БОЛЕЗНЬ
Korean 레이노 증후군
Croatian RAYNAUDOVA BOLEST
Polish Choroba Raynauda, Objaw Raynauda
Hungarian Raynaud, Raynaud-syndroma, Raynaud-betegség

Ontology: Raynaud Phenomenon (C0034735)

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.
Definition (NCI) 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 (AIR) WHAT: Raynaud's phenomenon. Raynaud's Phenomenon: the paroxysmal constriction of the small arteries and arterioles of the hands or feet, usually precipitated by cold or emotional upset, resulting in pallor and cyanosis of the fingers or toes following a characteristic pattern. WHY: Raynaud's phenomenon may occur in mixed connective tissue disease, systemic lupus erythematosus, progressive systemic sclerosis, poly- myositis/dermatomyositis, and rheumatoid arthritis associated with Sjogren's syndrome. HOW: In Raynaud's phenomenon there are three classic color changes of the fingers or toes. First, vasoconstriction results in a white blanching of the fingertips. Second, vasodilatation with sludging of vascular flow follows and results in blue, cyanotic digits. Finally, with recovery, there is increased blood flow with resulting erythema of the fingers. With observation of two of the three color changes, Raynaud's phenomenon is considered present. Local body cooling (by placing the hands in ice cold water) may demonstrate Raynaud's phenomenon, but some cases require general body cooling before the characteristic color phases occur. Permanent tissue damage can be induced by this testing, which therefore must be done only when absolutely necessary. If the digits show persistent cyanosis or there is evidence of pre-existing necrosis, performing this test is especially hazardous. One or more digits may be involved in Raynaud's phenomenon, and this involvement may be unilateral. REFS: 1) Spittell, JA: "Raynaud's phenomenon and allied vasospastic disorders". In Juergens, JL et al. (eds.): Peripheral Vascular Diseases, pp. 555-83. Philadelphia: W.B. Saunders Co., 1980. 2) Porter, JM; Snider, RL; Bardana, EJ; Rosch, J and Eidemiller, LR: The diagnosis and treatment of Raynaud's phenomenon. Surgery 77:11, 1975. DN19300-3.
Concepts Disease or Syndrome (T047)
MSH D011928
SnomedCT 266261006, 195296007, 73483003
English Raynaud's phenomenon (by history or observed), RAYNAUD PHENOMENON, RAYNAUD'S PHENOMENON, Raynauds Phenomenon, Raynaud's phenomenon (physical finding), Raynaud's phenomenon (diagnosis), raynaud's phenomenon, Raynaud's Syndrome, Raynauds' phen, Raynaud phenomenon, RAYNAUDS PHENOMENON, PHENOMENON, RAYNAUDS, phenomenon raynauds, phenomenon raynaud's, Raynauds phenomenon, raynaud phen, raynaud phenomenon, phen raynauds, raynauds' phen, Raynaud's phenomenon, Paroxysmal digital cyanosis, cyanosis; paroxysmal digital, Raynaud; phenomenon, paroxysmal; digital cyanosis, phenomenon; Raynaud, Raynaud Phenomenon, Raynaud's phenomenon, secondary, raynauds phenomenon, Raynaud's phenomenon (disorder), Raynaud's phenomenon (finding)
Spanish fenómeno de Raynaud (hallazgo), Raynaud's phenomenon, cianosis digital paroxística, fenómeno de Raynaud (concepto no activo), fenómeno de Raynaud (trastorno), fenómeno de Raynaud, Fenómeno de Raynaud
Italian Fenomeno di Raynaud
French Phén de Raynaud, Phénomène de Raynaud, PHENOMENE DE RAYNAUD, Syndrome de Raynaud
German Raynaud-Phaen, RAYNAUD PHAENOMEN, Raynaud Syndrom
Japanese レイノー現象, レイノーゲンショウ
Portuguese FENOMENO DE RAYNAUD, Fenómeno de Raynaud
Czech Raynaudův fenomén
Hungarian Raynau-phenomen, Raynaud-phenomen
Dutch Raynaud; fenomeen, cyanose; paroxysmaal digitaal, fenomeen; Raynaud, paroxysmaal; digitale cyanose, fenomeen van Raynaud