thromboangiitis obliterans buerger s disease n.
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Thromboangiitis Obliterans Buerger’s Disease. Nonatherosclerotic segmental inflammatory disease affecting small and medium-sized arteries/veins in upper/lower extremities Categorized as a vasculitis Highly inflammatory thrombus with sparing of vessel wall

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thromboangiitis obliterans buerger s disease
Thromboangiitis ObliteransBuerger’s Disease
  • Nonatherosclerotic segmental inflammatory disease affecting small and medium-sized arteries/veins in upper/lower extremities
  • Categorized as a vasculitis
    • Highly inflammatory thrombus with sparing of vessel wall
  • Most commonly seen in young men with heavy tobacco use
history
History
  • 1879 (von Winiwarter): first case of 57yo male with foot pain leading to gangrene
    • Pathologic specimen showed intimal proliferation, thrombosis, and fibrosis
    • Suggested that vessel changes distinct from atherosclerosis
  • 1908 (Buerger): detailed description of 11 amputated limbs at Mt. Sinai with endarteritis and endophlebitis
  • 1928 (Allen & Brown): 200 cases at Mayo Clinic
    • Jewish men that were heavy smokers
epidemiology
Epidemiology
  • More prevalent in Middle and Far East than in N. America
    • Mayo Clinic showed decline from 104/100k in ’47 to 12/100k in ’86
    • International series widely variable in terms of causes of limb ischemia
      • Western Europe 0.5-5.6%
      • Poland 3%
      • E.Germany 6.7%
      • Czech Republic 11.5%
      • Yugoslavia 39%
      • India 45-63%
  • Women have increasing incidence
    • Published series prior to 1970: 1-2%
    • 23% at Cleveland Clinic (1970-1987)
    • 19% at OHSU (1987)
etiology
Etiology
  • UNKNOWN!
    • Distinct from other vasculitis
      • 1. thrombus is highly cellular with less intense cellular reaction in vessel wall
      • 2. normal immunologic markers
  • Strong association with smoking
  • No gene association found yet
  • Conflicting studies regarding hypercoagulable states
    • Increased urokinase plasminogen activator
    • Impaired endothelium-dependent vasorelaxation
  • Immunologic mechanisms may be contributory
    • Increased cellular sensitivity to Types I and III collagen
pathology
Pathology
  • Inflammatory thrombosis that affects arteries and veins
    • Acute-phase
      • Inflammation involving all layers of vessel wall with occlusive thrombosis
      • Microabscesses & multinucleated giant cells
    • Intermediate phase
      • Progressive organization of occlusive thrombus
      • Prominent inflammatory infiltrate within thrombus
    • Chronic phase
      • Extensive recanalization
      • Adventitial & perivascular fibrosis
  • Segmental in distribution
    • Skip areas noted
    • Rare to involve cerebral, coronary, renal, or mesenteric vessels
  • Non-necrotizing involvement of vessel wall
clinical features
Clinical Features
  • Classic presentation
    • Young male smoker with onset of symptoms before age 40-45
    • Ischemia of distal small arteries and veins
  • Cleveland Clinic 1990: presenting signs/symptoms in 112 patients
  • Initial site of claudication is arch of foot
  • Usually >2 limbs involved
  • Not uncommon to see angiographic findings in asymptomatic limbs
  • Upper extremity involvement distinguished from atherosclerosis
clinical features1
Clinical Features
  • Classification Systems
    • Major Criteria
      • Onset of distal extremity ischemic symptoms prior to aqe 45
      • Tobacco abuse
      • Undiseased arteries proximal to brachial & popliteal
      • Objective documentation of distal occlusive disease by plethysmography
      • Exclusion of proximal embolic source, trauma, autoimmune disease, hypercoagulable state, atherosclerosis
    • Minor Criteria
      • Migratory superficial phlebitis
      • Raynaud’s syndrome
      • Upper extremity involvement
      • Instep claudication
  • No typical lab abnormalities
arteriography
Arteriography
  • Involvement of small and medium-sized vessels
    • Digital arteries of fingers and toes
    • Palmar, plantar, tibial, peroneal, radial, and ulnar
  • Segmental occlusive lesions
  • More severe disease distally
  • Corkscrew collaterals
  • Normal proximal arteries
treatment
Treatment
  • STOP ALL SMOKING!
    • Complete abstinence is the only way to stop progression of disease and prevent future amputation
  • All other therapies are palliative
    • Prostaglandin (iloprost)
    • Calcium channel blockers for vasospasm
    • Pentoxifylline
    • Sympathectomy
    • Thrombolytic therapy
    • Surgical revascularization
      • Limited due to skip lesions and distal disease
      • Usually <10% patients in series are bypass candidates
        • 5 year patency 49% in large series from Europe
          • 67% in those that stopped smoking and 35% in smokers
slide15

Nonatheroslerotic, segmental, inflammatory disease affecting small and medium sized arteries and veins of upper and lower extremities

  • Typically occurs in younger males with heavy tobacco use
  • Smoking cessation is key to therapy