Rheumatology Book

Environmental Injury

  • Raynaud's Phenomenon

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Raynaud's PhenomenonAka: Raynaud's Syndrome

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  1. 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
  2. Pathophysiology
    1. Exaggerated response to cold temperatures
    2. Increased Alpha Adrenergic Receptor responsiveness
    3. May be primary idiopathic or secondary cause
  3. 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
  4. 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)
  5. 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
  6. Signs
    1. Pallor or cyanosis of fingers or toes
    2. Thumb is not involved
  7. Evaluation
    1. Distal pulses
    2. Bruit Auscultation
    3. Signs of ischemia
    4. Allen's Test
    5. Assess for Thoracic Outlet Syndrome
  8. Labs
    1. Complete Blood Count (CBC)
    2. Serum Chemistry Panel (Chem7)
    3. Urinalysis
    4. Consider Antinuclear Antibody (ANA) when indicated
  9. Radiology
    1. Consider Arterial Doppler ultrasound
  10. 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
  11. Complications
    1. Cutaneous Ulceration of involved digit
    2. Gangrene
  12. 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
      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
  13. 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
  14. 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
  15. 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

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