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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