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
Most commonly seen in young men with
heavy tobacco use
 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
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
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
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
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
intermittent claudication 63%
rest pain 81%
Ischemic ulcers 76%
Thrombophlebitis 38%
sensory findings 69%
abnormal Allen’s test 63%

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 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
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
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
 Outcomes
Cleveland Clinic 1970-1996

120 patients

Smoking (n=68) Ex-smoking (n=52)

Amputation 43% No amputation 57% Amputation 6% No amputation 94%

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