II. Epidemiology
- Age of onset: Younger than 45 years old
- Men predominate
-
Prevalence (Asian and eastern European descent)
- United States: 12%
- India: 63%
- Ashkenazi Jews in Israel: 80%
III. Pathophysiology
- Nonatherosclerotic inflammatory disease
- Affects small and medium sized extremity vessels
IV. Risk factors
- Tobacco Abuse is highly correlated
V. Symptoms
- Raynaud's Phenomenon
- Ischemia of distal extremities (hands and feet)
- Claudication
- Rest pain
- Ulcerations of toes and fingers
VI. Signs
- Distal pulses diminished
- Radial pulse and ulnar pulse decreased
- Posterior tibial and dorsalis pedis decreased
- Proximal pulses full
- Brachial pulses normal
- Popliteal pulses normal
VII. Labs: Rule-out other causes of Vasculitis or thrombosis
- Acute phase reactants normal
- Erythrocyte Sedimentation Rate normal
- C-Reactive Protein normal
- Complete Blood Count normal
- Liver Function Tests normal
- Serum Creatinine normal
- Fasting Glucose normal
- Autoimmune markers negative
- Antinuclear Antibody (ANA) not present
- Rheumatoid Factor (RF) not present
- CREST syndrome markers not present
- Scleroderma markers not present
- Hypercoagulable state markers negative
VIII. Diagnostic
- Biopsy
- Inflammatory, highly cellular occlusive thrombus
- Angiography
- Severe distal segmental vessel Occlusion
IX. Management
- Tobacco Cessation
- Aspirin
- Iloprost IV (not available in United States)
X. References
- Olin (2000) N Engl J Med 343:864-9 [PubMed]
- Wilson (1991) Harrison's Medicine, p. 1020