What is Buerger's disease?

What is Buerger's Disease?

Buerger's disease (thromboangiitis obliterans) is a nonatherosclerotic inflammatory vasculitis that causes segmental thrombotic occlusions of small- and medium-sized arteries and veins in the distal extremities, almost exclusively affecting young tobacco smokers under age 50. 1

Pathophysiology and Etiology

• Buerger's disease is an arterial obliterative and thrombotic process that behaves like a vasculitis, affecting arteries of all sizes (smaller distal limb arteries more frequently than larger proximal arteries) as well as superficial veins. 1

• The disease is thought to be an immune-mediated endarteritis, with immunocytochemical studies identifying linear deposition of immunoglobulins and complement factors along the elastic lamina, though the inciting antigen remains unknown. 2

• Acute histopathology shows intensely cellular vessel wall inflammation, giant cell foci, and hypercellular thrombi, but with preservation of the elastic lamina and overall vascular wall architecture—distinguishing it from atherosclerosis and necrotizing arteritis. 2

• Tobacco abuse is inextricably linked to disease onset and progression, though it appears to be a synergistic factor triggering an autoimmune response rather than the sole cause. 2, 3

Clinical Presentation

• Patients are typically young (onset before age 45-50), predominantly male smokers who present with distal extremity ischemia, ischemic ulcers, or frank gangrene of the toes or fingers. 1, 2, 3

• The incidence is approximately 12.6 per 100,000 in North America. 1

• Clinical manifestations include:

  • Intermittent claudication (23% of patients) 4
  • Rest pain (13% of patients) 4
  • Ulceration or gangrene (64% of patients) 4
  • Upper limb involvement including cyanosis, paleness, and gangrene (84% of patients) 4
  • Superficial thrombophlebitis/phlebitis migrans (34% of patients) 4

• Large arteries are typically spared, as are the coronary, cerebral, and visceral circulations, though arterial lesions can extend to femoral arteries (25%), iliac arteries (8%), or even abdominal aorta/visceral arteries (6%) in some cases. 2, 4

Diagnostic Criteria

The diagnosis of Buerger's disease is based on clinical criteria and is a diagnosis of exclusion. 3, 5 The traditional Shionoya criteria require all five of the following:

1. History of smoking or tobacco abuse 2, 3, 4
2. Age of onset less than 45-50 years 2, 3, 4
3. Infrapopliteal segmental arterial occlusions with sparing of proximal vasculature 2, 3
4. Either upper limb involvement (Raynaud's syndrome or digital ulceration) or phlebitis migrans 2, 3, 4
5. Exclusion of atherosclerosis, diabetes, true arteritis, proximal embolic source, hypercoagulable states, and other cardiovascular risk factors 2, 3, 4

Imaging Findings

• Arteriography classically demonstrates "corkscrew," "spider legs," or "tree roots" collateral vessels representing pathologically dilated vasa vasorum—these findings are suggestive but not pathognomonic. 1, 3, 5

• There is no specific diagnostic test or positive serologic markers for Buerger's disease. 3

Treatment and Prognosis

Total abstinence from tobacco use remains the only means of stopping disease progression and is the mainstay of treatment. 2, 3, 5

• Tobacco abstinence generally results in disease quiescence. 2

• Initial management should be conservative, with claudicants encouraged to walk and patients with critical ischemia admitted for bed rest. 3

• Traditional medical treatments are largely ineffective, and endovascular or open surgical approaches are often not possible due to the small caliber of affected vessels and lack of suitable distal targets or venous conduits. 6, 3

• Bypass grafting is seldom an option; when performed (mainly femorodistal bypasses), patency rates are suboptimal though limb salvage rates can be satisfactory. 3

• Minor limb amputations are required in approximately 20% of patients, and major limb amputations in 4%. 4

• While mortality is not increased, patients often suffer from severe ischemic pain and tissue loss. 2

Emerging Considerations

• Several studies suggest oral bacterial infections (severe periodontitis) may activate disease onset, with more than half of patients suffering from severe periodontitis. 4

• Therapeutic angiogenesis techniques using gene- and cell-based therapies are under investigation for patients with critical limb ischemia who are not candidates for traditional revascularization. 6