Description

• Nonatherosclerotic vasculitis of small- and medium-sized arteries and veins resulting in segmental occlusion
• Characterized clinically by an inflammatory and vaso-occlusive phenomenon, rest pain, unremitting ischemic ulcerations, and gangrene of the digits of hands and feet
• Occurs primarily in men who smoke
• System(s) affected: Cardiovascular
• Synonym(s): Buerger disease

Epidemiology

• The prevalence has decreased in North America over the last 30 years.
• Worldwide, but most prevalent in Eastern Europe, Mediterranean, and Asian countries

• 11–30/100,000 persons/year
• Predominant age: 20–40 years
• Predominant sex: Male > Female; increasing numbers of women are being diagnosed, possibly due to increased smoking (1).

• Estimates range from as low as 0.5–5.5% in Western Europe, to 45–63% in India, to 80% in Israel among Jews of Ashkenazi ancestry.
• Accounts for 5% and 16% of patients hospitalized for arterial occlusive disease in Europe and Japan, respectively
• 13/100,000 US population
• Overall occurrence is decreasing worldwide (2).

Geriatric Considerations
Not common in this age group

Pediatric Considerations
Should be considered in the differential diagnosis of the young patient with claudication.

Risk Factors

• Smoking tobacco, with rare cases with smokeless tobacco/snuff. The degree of dependence is similar to that in subjects with coronary artery disease.
• Chronic anaerobic periodontal infection also may play a role in the development of Buerger disease.

• Greater prevalence of HLA-A54, HLA-A9, and HLA-B5
• HLA-B12 antigen may be associated with disease resistance.
• Familial cases reported rarely

General Prevention

Never smoke. Tobacco and smoking cessation is the only way to prevent Buerger disease.

Pathophysiology

• Impaired endothelium-dependent vasorelaxation and decreased peripheral sympathetic outflow
• Segmental infiltration of inflammatory cells in vessel wall leads to thrombotic occlusion of vessel.
• Highly cellular and inflammatory thrombus with relative sparing of the blood vessel wall

Etiology

• Idiopathic
• Smoking
• Genetic factors
• Autoimmune disorder with cell-mediated sensitivity to types I and III human collagens (both are normal constituents of blood vessels)
• Impaired peripheral endothelium-dependent vasodilation. Nonendothelial mechanisms of vasodilation are intact.
• Arsenic content of tobacco
• Chronic anaerobic periodontal infection