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Updated: Dec 8 2021

Buerger Disease (Thromboangiitis Obliterans)

  • 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
    • Associated conditions
      • Raynaud phenomenon
  • 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
  • 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
  • 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
    • Making the diagnosis
      • based on a history of current or recent tobacco use and clinical presentation
  • 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
  • Prognosis
    • Typically resolves with cessation of smoking
    • Patients who do not stop smoking often eventually undergo amputation
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