II. 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

III. Pathophysiology

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

IV. Causes: 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. Thromboangiitis Obliterans (Buerger's Disease)
  4. Medications
    1. See provocative factors below
  5. Hyperviscosity state (e.g. Polycythemia Vera)
  6. Paraproteinemia
  7. Cryoglobulinemia

V. Causes: Provocative Factors

VI. 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 is more common and benign
  3. Sensation of numbness, clumsiness or "pins and needles"
  4. One finger may be more sensitive than the others

VII. Signs

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

VIII. Evaluation

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

IX. Labs

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

X. Imaging

  1. Consider Arterial Doppler Ultrasound

XI. 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

XII. Complications

  1. Cutaneous ulceration of involved digit
  2. Gangrene

XIII. Management: First Line

  1. Conservative Measures
    1. Smoking Cessation
    2. Avoid precipitating medications (see above)
    3. Limit Caffeine
    4. 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 ER (Procardia) orally daily
    2. Amlodipine
  3. Other medications
    1. Sildenafil (Viagra)
    2. Nitroglycerin Ointment (Nitro-Bid 2%)
      1. Apply to affected fingers or toes four times daily
      2. Do not use with Sildenafil (risk of Hypotension)
    3. Sodium Nitrate with Ascorbic Acid gel
      1. Tucker (1999) Lancet 354:1670-5 [PubMed]

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

  1. See first-line measures above
  2. Consider Thromboangiitis Obliterans (Buerger's Disease)
  3. Alpha-adrenergic blockers
  4. Angiotensin Receptor Blockers
  5. Pentoxifylline (Trental)

XV. 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

XVI. 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

XVII. References

  1. (2018) Presc Lett 25(12): 70
  2. Oreizi-Esfahani (1996) Consultant, p. 905-12
  3. Wigley (1999) Consultant p. 540-54
  4. Comfort-Adee (1993) Am Fam Physician, 47(4): 823-9 [PubMed]

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