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BUERGERS DISEASE

Satria Pandu Persada Isma

Background

Also known as thromboangiitis obliterans Nonatherosclerotic, segmental, inflammatory, vasoocclusive disease Affects the small- and medium-sized arteries and veins of the upper and lower extremities Strongly associated with heavy tobacco use.

Patophysiology

Etiology is unknown Exposure to tobacco is essential for both initiation and progression of the disease Immunologic phenomenon that leads to vasodysfunction and inflammatory thrombi Prevalence of HLA-A9, HLA-A54, and HLA-B5 is observed.

Frequency

12.6-20 cases per 100,000 population (US) More common in males (M:F ratio 3:1)

Most patients are aged 20-45

History

Age younger than 45 years

Current (or recent) history of tobacco use


Presence of distal extremity ischemia Exclusion of autoimmune diseases, hypercoagulable states, and diabetes mellitus Exclusion of a proximal source of emboli

Consistent arteriographic findings

History (contd.)

70-80%present with distal ischemic rest pain and/or ischemic ulcerations on the toes, feet, or fingers Involvement of large arteries is unusual May also present with claudication of the feet, legs, hands, or arms and often describe the Raynaud phenomenon May present with foot infections

Physical Exam

Develop painful ulcerations and/or frank gangrene of the digits Hands and feet are usually cool and mildly edematous Superficial thrombophlebitis (often migratory) Paresthesias Impaired distal pulses 80% percent of patients present with involvement of 3-4 limbs.

Physical Exam (contd.)


Determine

the colour Temperature Vascular angle Cappilary refilling Capillary filling time Feel all the pulses Venous filling Auscultate Pressure areas Check all the nerves Allens test

Differentials

Raynaud phenomenon Systemic lupus erythematosus Antiphospholipidantibody syndrome Diabetes mellitus

Carpal tunnel syndrome Peripheral neuropathy Neurotrophic ulcers Trauma Vasculitis, other causes

Atherosclerosis

Laboratory Workup

No specific laboratory tests confirm or exclude the diagnosis of Buerger disease Primary goal of a laboratory workup in patients thought to have the disease is to exclude other disease processes

Imaging Studies

Angiography/arteriography
nonatherosclerotic, segmental occlusive lesions of the small- and medium-sized vessels corkscrew collaterals

Echocardiography

Morphology
Segmental acute & chronic vasculitis with secondary spread to contiguous veins and nerves Inflammation permeates arterial walls accompanied by thrombosis of the lumen Characteristically the thrombus contains microabscesses marked by a central focus of neutrophils surrounded by granulomatous inflammation

Medical Care
Absolute discontinuation of tobacco use is the only strategy proven Use of thrombolytic therapy remains inconclusive Intravenous iloprost ? Use of well-fitting protective footwear Avoidance of cold environments Avoidance of drugs that lead to vasoconstriction

Surgical Treatment

Surgical revascularization for Buerger disease is usually not feasible Autologous vein bypass Sympathectomy Amputation

Diet & Activity

No dietary restrictions are needed Encourage cardiovascular exercise, activity should be restricted by symptoms only

Complications

Ulcerations Gangrene Need for amputation

Rare occlusion of cerebral, coronary, renal, splenic, or mesenteric arteries

Prognosis

Among patients with who quit smoking, 94% avoid amputation Patients who continue smoking there is a 43% chance that an amputation will be required sometime during a 7-8 year period Mortality is rare

GRAZIE

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