Updated: 9/30/2019

Buerger Disease (Thromboangiitis Obliterans)

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Snapshot
  • A 30-year-old man presents to his physician’s office for intermittent pain in his hands and feet and discoloration of 3 of his fingers, which he says is painful. He has had pain in his extremities for about 1 year now, but this is the first time he’s seen discoloration of his fingers. He has a 20-pack-year smoking history. 
Introduction
  • Clinical definition
    • an inflammatory vasculitis characterized by claudication from vaso-occlusion
      • also known as thromboangiitis obliterans
  • Epidemiology
    • demographics
      • male > female
        • 3:1 ratio
      • common in adults < 40 years of age
    • risk factors
      • smoking is the strongest risk factor
  • Pathogenesis
    • primarily a medium vessel vasculitis, but the disease can also affect the small vessels 
    • the disease is thought to be due to a combination of immunologic hypersensitivity to tobacco extracts, defects in endothelium-dependent vasodilation, and anti-endothelial cell antibodies
  • Associated conditions
    • Raynaud phenomenon
  • Prognosis
    • typically resolves with cessation of smoking
    • patients who do not stop smoking often eventually undergo amputation
Presentation
  • Symptoms
    • intermittent claudication and pain in the feet or hands
    • numbness or tingling
    • may have autoamputation of digits
    • Raynaud phenomenon
  • Physical exam
    • superficial thrombophlebitis
      • often migratory
      • distended veins with redness and tenderness
      • darkened or discolored skin
    • gangrene
    • cool extremities
    • limb ischemia
      • pain, paresthesia, pallor, mottling, pulselessness, and poikilothermia
    • ischemic ulcers, especially in the toes or fingers
Imaging
  • Echocardiography
    • indication
      • for all patients to exclude another source of distal vessel occlusion
Studies
  • Laboratory evaluation should be used to exclude other autoimmune diseases
  • Arteriography
    • indication
      • diagnostic
    • findings
      • non-atherosclerotic segmental occlusion of primarily medium-sized vessels, but also small-sized vessels
      • corkscrew collaterals found around the area of occlusion
  • Biopsy of involved vessels
    • indication
      • performed only if the diagnosis is uncertain
  • Histology
    • segmental thrombosing vasculitis
  • Making the diagnosis
    • based on a history of current or recent tobacco use and clinical presentation
Differential
  • Raynaud phenomenon
    • distinguishing factor
      • typically does not result in ulcers, gangrene, or need for amputation
  • Polyarteritis nodosa
    • distinguishing factor
      • often is accompanied by systemic symptoms such as fever or weight loss and is not associated with smoking
Treatment
  • Management approach
    • Buerger disease and its progression are strongly associated with smoking use
      • therefore, management is aimed at smoking cessation, and pharmacologic therapy is ineffective
  • Conservative
    • smoking cessation
      • indication
        • for all patients
    • activities to increase circulation
      • indication
        • for all patients
      • modalities
        • apply warmth
        • exercise
  • Operative
    • amputation
      • indications
        • nonhealing ulcers
        • gangrene
        • pain refractory to all other measures
Complications
  • Sepsis
  • Need for amputation

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