Post on 14-Apr-2018
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Thromboangiitis Obliterans
Buergers Disease
Nonatherosclerotic segmentalinflammatory disease affecting small andmedium-sized arteries/veins in
upper/lower extremitiesCategorized as a vasculitis
Highly inflammatory thrombus with sparing ofvessel wall
Most commonly seen in young men withheavy tobacco use
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History
1879 (von Winiwarter): first case of 57yo malewith foot pain leading to gangrene Pathologic specimen showed intimal proliferation,
thrombosis, and fibrosis
Suggested that vessel changes distinct fromatherosclerosis
1908 (Buerger): detailed description of 11amputated limbs at Mt. Sinai with endarteritis
and endophlebitis
1928 (Allen & Brown): 200 cases at Mayo Clinic Jewish men that were heavy smokers
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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
ischemiaWestern 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)
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Etiology
UNKNOWN! Distinct from other vasculitis
1. thrombus is highly cellular with less intense cellular reaction invessel 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
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Pathology
Inflammatory thrombosis that affects arteries and veins Acute-phase
Inflammation involving all layers of vessel wall with occlusivethrombosis
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
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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
intermittent claudication 63%
rest pain 81%
Ischemic ulcers 76%
Thrombophlebitis 38%
sensory findings 69%
abnormal Allens test 63%
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Clinical Features
Classification Systems Major CriteriaOnset of distal extremity ischemic symptoms prior to aqe 45
Tobacco abuse
Undiseased arteries proximal to brachial & popliteal
Objective documentation of distal occlusive disease byplethysmography
Exclusion of proximal embolic source, trauma, autoimmunedisease, hypercoagulable state, atherosclerosis
Minor CriteriaMigratory superficial phlebitis
Raynauds syndromeUpper extremity involvement
Instep claudication
No typical lab abnormalities
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Arteriography
Involvement of small and medium-sizedvessels
Digital arteries of fingers and toes
Palmar, plantar, tibial, peroneal, radial, andulnar
Segmental occlusive lesions
More severe disease distallyCorkscrew collaterals
Normal proximal arteries
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Treatment
STOP ALL SMOKING! Complete abstinence is the only way to stopprogression of disease and prevent future amputation
All other therapies are palliative Prostaglandin (iloprost)
Calcium channel blockers for vasospasm Pentoxifylline
Sympathectomy
Thrombolytic therapy
Surgical revascularizationLimited due to skip lesions and distal disease
Usually
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OutcomesCleveland Clinic 1970-1996
120 patients
Smoking (n=68) Ex-smoking (n=52)
Amputation 43% No amputation 57% Amputation 6% No amputation 94%
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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