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)
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
| 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
