ISCHEMIC STROKE / CLASSIFICATION AND ETIOLOGY

Thromboangiitis obliterans (Buerger´s disease)

David Goldemund M.D.
Updated on 29/01/2024, published on 10/05/2023

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 extremities
cerebrovascular involvement is rare but has been reported
the disease predominantly affects young men under 45 years of age who are heavy smokers or chew tobacco (the prevalence among female smokers is low)
- the average age for the onset of symptoms is around 35 years
inflammation leads to arterial stenosis and potential thrombosis, causing pain, tissue damage, and even gangrene (in severe cases, amputation may be required)
quitting smoking is the most important step in preventing and managing the disease

Etiopathogenesis

the exact cause and pathogenesis remain unclear
there is strong evidence indicating 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)
this 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 or exercise
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; gangrene may occur in severe cases
although CNS symptoms (including stroke) have been sporadically reported, they are not common (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

Biopsy

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
multilimb involvement with a predilection for the lower extremities
typical extensive obliteration of small and medium-sized vessels + thin corkscrew-like collaterals
normal findings in proximal arterial segments without atherosclerotic changes

Buerger's disease on DSA

Buerger's disease - corkscrew-like collaterals

A Scoring System for the Clinical Diagnosis of Buerger's Disease

Positive points
Age at onset	<30	+2
Age at onset	30-40	+1
Foot intermittent claudications	present	+2
Foot intermittent claudications	by history	+1
Upper extremity	symptomatic	+2
Upper extremity	asymptomatic	+1
Migrating superficial venous thrombosis (SVT)	present	+2
Migrating superficial venous thrombosis (SVT)	by history	+1
Raynaud color changes	present	+2
Raynaud color changes	by history	+1
Angiography	typical	+1
Biopsy	typical	+1

Negative points
Age at onset	45-50	-1
Age at onset	> 50	-2
Sex, smoking	female	-1
Sex, smoking	non-smoker	-2
Location	single limb	-1
Location	no lower extremities involved	-2
Absent pulse	brachial	-1
Absent pulse	femoral	-2
Vascular risk factors (diabetes, HT, etc.)	discovered after diagnosis 5-10 years	-1
Vascular risk factors (diabetes, HT, etc.)	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 effective measure to prevent the worsening of Buerger’s disease is to stop smoking → smoking cessation
no significant effect of antiplatelet, anticoagulant, or steroid therapy has been demonstrated
avoid cold temperatures and other conditions that reduce blood flow to the limbs
treat infections promptly, use anti-inflammatory drugs for phlebitis, or consider vasodilators
the efficacy of immunoadsorption is under discussion
revascularization options are limited; sympathectomy may be considered in certain cases
amputation is necessary if gangrene develops

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Thromboangiitis obliterans (Buerger´s disease)
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