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Updated: Feb 5 2019

Raynaud Phenomenon

  • Snapshot
    • A 50-year-old man presents to his physician’s office for discolored fingers. He reports that this happens in the cold or when he is emotionally stressed. As he is going through a divorce, he reports this happening more often than not. He reports that there is no pain, but he sometimes feels tightening in his fingers with the discoloration. He has a past medical history of limited scleroderma as well as a family history of Raynaud phenomenon. His physical exam is notable for blue fingertips. He is prescribed a calcium channel blocker for his symptoms.
  • Introduction
    • Clinical definition
      • discoloration in extremities due to exaggerated physiologic response to cold temperatures or emotional stress
      • classification
        • Raynaud disease
          • primary (idiopathic) Raynaud phenomenon is not associated with an underlying disease
        • Raynaud syndrome
          • secondary Raynaud phenomenon is associated with an underlying disease
    • Pathogenesis
      • ↓ blood flow to skin due to exaggerated physiologic response to cold temperatures or emotional stress
        • small vessel vasospasm with impaired vasodilation and increased vasoconstriction
      • phases
        • initially, extremities turn white, indicating ischemia
        • then, extremities turn blue, indicating hypoxia and cyanosis
        • finally, extremities turn pink with re-warming, indicating reperfusion
    • Associated conditions
      • systemic sclerosis or limited scleroderma
      • systemic lupus erythematosus
      • mixed connective tissue disease
      • cryoglobulinemia
      • thromboangiitis obliterans (Buerger disease)
      • juvenile rheumatoid arthritis
  • Epidemiology
    • Demographics
      • Raynaud disease
        • majority of patients with Raynaud phenomenon
        • female > male
        • most commonly < 30 years of age
      • Raynaud syndrome
        • most commonly > 30 years of age
    • Location
      • fingers > toes
    • Risk factors
      • family history
      • autoimmune disease
  • Etiology
    • cold temperatures
    • emotional stress
    • drugs
      • beta blockers
  • Presentation
    • Symptoms
      • attacks of triphasic color changes in extremities with cold temperatures or emotional stress
        • white, blue, then pink or red
        • white and blue phase may be associated with feeling of tightness or pain in severe disease
    • Physical exam
      • may not present with all 3 phases
      • usually in fingers or toes, but can also affect nose, cheeks, or ears
      • may have faint peripheral pulses
      • may have digital ischemia, but this is uncommon
        • ulcers
        • gangrene
  • Studies
    • Labs
      • only indicated if an underlying disease is suspected
    • Making the diagnosis
      • most cases are clinically diagnosed
      • can use cold stimulation test to trigger an attack
        • digits rewarm in > 20 minutes if the patient has Raynaud phenomenon
  • Differential
    • Thromboangiitis obliterans (Buerger disease)
      • distinguishing factor
        • a vasculitis that results in digital necrosis and ischemia
    • Frostbite
      • distinguishing factors
        • the disease does not resolve with rewarming
        • when thawed, patients may have bullae with clear or hemorrhagic fluid
  • Treatment
    • Conservative
      • avoid cold temperatures
        • indication
          • for all patients
        • modalities
          • use of mittens or gloves
      • smoking cessation
        • indication
          • for all patients
    • Medical
      • dihydropyridine-type calcium channel blockers
        • indication
          • for patients refractory to conservative methods
        • drugs
          • nifedipine, nimodipine, nicardipine, or amlodipine
          • dihydropyridines act on vascular smooth muscle
      • phosphodiesterase inhibitors
        • indication
          • for patients refractory to calcium channel blockers
        • drugs
          • sildenafil, vardenafil, or tadalafil
  • Complications
    • Digital ulceration
      • more common with secondary Raynaud syndrome
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