Buerger’s Disease

What is Buerger’s Disease?

Buerger’s or thromboangiitis obliterans is a non-atherosclerotic, segmental, inflammatory and clotting disease that mostly affects the small to medium-sized arteries and veins of the extremities, and leads to decrease or loss of blood supply (ischemia) distally, and in severe cases may cause ulcers and gangrene.

What causes Buerger’s Disease?

Although smoking tobacco is central to the initiation, continuation and recurrence of disease, the specific role of smoking in Buerger’s is unknown. It is postulated that smoking may cause allergic, toxic or autoimmune reaction that leads to the chronic inflammation and clotting. Endothelial (inner layer of a vessel) dysfunction as well as high coagulable state and genetic factors may also play a role in the disease process.

Who is mostly affected?

Buerger’s is more common in countries where tobacco is heavily used (Mediterranean, Middle East andAsia), especially among people who make homemade cigarettes from raw tobacco. Men are more commonly affected than women and the typical age of onset is 40 to 45 years. However, there are reports of increasing prevalence of disease in women, possibly due to the increasing use of cigarettes among women.

Symptoms

Hands or feet pain that can be severe, mostly during exercise and sometimes at rest, is caused by limited blood supply. Patient may also experience limb tingling and numbness. In severe cases, fingers and toes ulceration and gangrene may occur and lead to limb loss. Raynaud’s phenomenon (white then purple discoloration of the affected limb upon cold exposure) and thrombophlebitis (superficial vein clot) have also been described.

Diagnosis

Diagnosis is based on the clinical history, exam findings, and vascular tests proving the evidence of ischemia in the absence of atherosclerosis and other etiologies.  There is no specific laboratory or test to diagnose Buerger’s. However, vascular laboratory studies, blood vessel studies with computed tomography (CT) or magnetic resonance angiogram (MRA) or conventional angiogram can help making the diagnosis and excluding other etiologies. Biopsy is rarely needed.

Treatment

Smoking cessation is the only definitive therapy. Medications that can dilate the vessels, like calcium channel blockers and prostaglandin analogues, can be tried. Application of intermittent pneumatic compression can help augmenting the arterial blood flow to the affected limb. Surgical revascularization is usually not indicated due to the distal nature of occlusive disease and because most patients do well with smoking cessation.

Prognosis

Tobacco abstinence is essential to stop the progression of the disease.

Revision: September 2012

The Vasculitis Foundation gratefully acknowledges Dr. Nedaa Shiek from the Mayo Clinic, Rochester, MN, for his expertise and contribution in compiling this information.

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