Thromboangiitis obliterans (Buerger's disease)

Definition

Thromboangiitis obliterans (Buerger's disease) is an inflammatory thrombosis of small and medium-sized arteries and some superficial veins. It causes arterial ischemia in the distal extremities and superficial thrombophlebitis. The main risk factor is tobacco use.

Occurrence & epidemiology

The disease occurs almost exclusively in people who use tobacco (nearly all of them smoke). It predominantly affects men aged 30 to 45 years and only rarely women. It is most common in Asia and the Middle East, where tobacco use is heavy.

Aetiopathogenesis

The cause is unknown; cigarette smoking is the main risk factor. Oxidative stress is involved in the mechanism, possibly also delayed hypersensitivity or toxic angiitis. According to another theory, it is an autoimmune disease with cell-mediated sensitisation to type I and III collagen of the vessel wall.

The disease causes segmental inflammation of small and medium-sized arteries and frequently of superficial veins of the extremities.

Clinical features

The symptoms are those of arterial ischemia and superficial venous thrombosis. Some patients have migratory phlebitis (thrombophlebitis migrans), usually in superficial veins of the foot or leg.

• gradual onset in the most distal vessels of the arms and legs with coldness, numbness, tingling or burning, sometimes before objective findings
• frequently Raynaud's phenomenon
• claudication, usually in the arch of the foot or the leg, rarely in the hand, arm or thigh; progression to rest pain
• with severe pain, a cold, sweating, cyanotic limb
• later ischemic ulcers progressing to gangrene, usually of one or more fingers or toes
• weak or absent pulses in the foot arteries, often also at the wrist
• pallor on elevation and redness when dependent

Histology

• Acute stage: occlusive thrombi with infiltration of the intima by neutrophils and lymphocytes; endothelial cells proliferate, but the internal elastic lamina remains intact
• Intermediate stage: the thrombi organize and recanalise incompletely; the media is preserved but may be infiltrated by fibroblasts
• Older lesions: periarterial fibrosis, sometimes also involving the adjacent vein and nerve

Diagnosis

History and findings suggest the diagnosis; other causes of ischemia are excluded by testing.

• Allen test: in young men who smoke and have extremity ulcers, a positive test (the hand remains pale after compression and alternating release of the radial and ulnar arteries) suggests the disease
• Ankle-brachial index or segmental pressure measurement: demonstration of distal ischemia; markedly reduced blood flow and pressures in the toes, feet and fingers
• Echocardiography: exclusion of cardiac emboli
• Laboratory tests: ANA, rheumatoid factor, complement, anticentromere and anti-Scl-70 antibodies to exclude vasculitis or connective tissue disease; antiphospholipid antibodies to exclude antiphospholipid syndrome (may be slightly raised in thromboangiitis obliterans)
• Angiography: typical are segmental occlusions of distal arteries in the hands and feet, tortuous corkscrew collaterals around the occlusions and absence of atherosclerosis

Keep learning in the app

Further reading (open access)

1. MSD Manual Professional: Thromboangiitis Obliterans
2. StatPearls: Buerger Disease

Cross-references