II. Epidemiology

  1. Age of onset: Younger than 45 years old
  2. Men predominate
  3. Prevalence (Asian and eastern European descent)
    1. United States: 12%
    2. India: 63%
    3. Ashkenazi Jews in Israel: 80%

III. Pathophysiology

  1. Nonatherosclerotic inflammatory disease
  2. Affects small and medium sized extremity vessels

IV. Risk factors

  1. Tobacco Abuse is highly correlated

V. Symptoms

  1. Raynaud's Phenomenon
  2. Ischemia of distal extremities (hands and feet)
    1. Claudication
    2. Rest pain
    3. Ulcerations of toes and fingers

VI. Signs

  1. Distal pulses diminished
    1. Radial pulse and ulnar pulse decreased
    2. Posterior tibial and dorsalis pedis decreased
  2. Proximal pulses full
    1. Brachial pulses normal
    2. Popliteal pulses normal

VII. Labs: Rule-out other causes of Vasculitis or thrombosis

  1. Acute phase reactants normal
    1. Erythrocyte Sedimentation Rate normal
    2. C-Reactive Protein normal
  2. Complete Blood Count normal
  3. Liver Function Tests normal
  4. Serum Creatinine normal
  5. Fasting Glucose normal
  6. Autoimmune markers negative
    1. Antinuclear Antibody (ANA) not present
    2. Rheumatoid Factor (RF) not present
    3. CREST syndrome markers not present
    4. Scleroderma markers not present
  7. Hypercoagulable state markers negative

VIII. Diagnostic

  1. Biopsy
    1. Inflammatory, highly cellular occlusive thrombus
  2. Angiography
    1. Severe distal segmental vessel Occlusion

IX. Management

  1. Tobacco Cessation
  2. Aspirin
  3. Iloprost IV (not available in United States)

X. References

  1. Olin (2000) N Engl J Med 343:864-9 [PubMed]
  2. Wilson (1991) Harrison's Medicine, p. 1020

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