Thromboangiitis Obliterans Buerger's Disease

Thromboangiitis Obliterans Buerger's Disease

Thromboangiitis Obliterans Buergers 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 Allens 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 Raynauds 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) Amputation 43% No amputation 57% Ex-smoking (n=52) 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

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