ISCHEMIC STROKE / CLASSIFICATION AND ETIOLOGY

Thromboangiitis obliterans (Buerger´s disease)

Created 10/05/2023, last revision 20/07/2023

Definition

  • Buerger’s disease (also known as thromboangiitis obliterans) is a nonatherosclerotic segmental inflammatory disease that most commonly affects small and medium-sized arteries and veins of the upper and lower limbs
  • cerebrovascular involvement is rare but has been reported
  • the disease usually affects young males under 45 who are heavy smokers or chew tobacco (the prevalence among female smokers is low)
    • the average age when symptoms appear is around 35
  • inflammation leads to arterial stenosis and potential thrombosis, causing pain, tissue damage, and even gangrene (in severe cases, amputation may be required)

Etiopathogenesis

  • the exact cause and pathogenesis are unclear
  • there is strong evidence that smoking is a major contributor
  • an inflammatory process with granulomas occurs in peripheral small and medium-sized arteries and veins
    • elevated pro- and anti-inflammatory cytokines and various kinds of autoantibodies have been identified in patients with TAO (Kaiser, 2021)
  • extensive stenotic process and thrombosis lead to limb ischemia

Clinical presentation

  • fingers and toes are pale or bluish and feel cold to the touch; progressive necrosis may occur
  • pain in hands or feet at rest
    • pain may feel like a burning or tingling sensation
    • pain may worsen when the hands and feet get cold or during emotional stress
  • intermittent claudication – pain in the legs, ankles, or feet (arch of the foot) when walking
  • skin changes, incl. small painful sores on the fingers or toes
  • CNS symptoms (including stroke) have been sporadically reported   (Kaiser, 2021)
Gangrene in Buerger's disease

Diagnostic evaluation

Diagnosis is based on typical clinical presentation, vascular imaging, and patient characteristics (age, smoking history)

Blood tests

  • there is no specific blood test to diagnose thromboangiitis obliterans.
  • it is necessary to exclude other vasculitides
  • in rare cases, a biopsy may be performed
    • the gold standard to establish a definitive diagnosis
    • acute phase histology of the affected superficial vein shows a hypercellular, inflammatory thrombus with less pronounced inflammatory changes in the vessel wall

Vascular imaging

  • ultrasound, CTA, MRA, or DSA may be used
  • multi-limb involvement with a predilection for the lower limbs
  • typical extensive obliteration of small and medium-sized vessels + thin corkscrew-like collaterals
  • normal findings in proximal arterial segments with no atherosclerotic changes
Buerger's disease on DSA
Buerger's disease - corkscrew-like collaterals
Positive points
Age at onset
<30 +2
30-40 +1
Foot intermittent claudications
present +2
by history +1
Upper extremity
symptomatic +2
asymptomatic +1
Migrating superficial venous thrombosis (SVT)
present +2
by history +1
Raynaud color changes
present +2
by history +1
Angiography typical +1
Biopsy typical +1
Negative points
Age at onset
45-50 -1
> 50 -2
Sex, smoking
female -1
non-smoker -2
Location
single limb
-1
no lower extremities involved
-2
Absent pulse
brachial -1
femoral -2
Vascular risk factors (diabetes, HT, etc.)
discovered after diagnosis  5-10 years -1
2-5 years -2
Points  probability of diagnosis
0-1 diagnosis excluded
2-3 suspected, low probability
4-5 probable, medium probability
≥ 6
definite, high probability

Management

  • no evidence-based causal therapy; the only way to keep Buerger’s disease from getting worse is to stop using tobacco  → smoking cessation
  • no significant effect of antiplatelets, anticoagulants, or steroid therapy has been demonstrated
  • avoid cold temperatures and other conditions that reduce blood flow to the limbs
  • treat infections, use anti-inflammatory drugs for phlebitis, or use vasodilators
  • the efficacy of immunoadsorption is being discussed
  • revascularization options are limited; sympathectomy may be considered
  • amputation is necessary if gangrene develops

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Thromboangiitis obliterans (Buerger´s disease)
link: https://www.stroke-manual.com/thromboangiitis-obliterans-buerger-disease/