Buerger's Disease (Thromboangiitis Obliterans)

What happens in Buerger's disease

Inflammation develops in the walls of small and medium-sized arteries and veins, especially in the lower legs, feet, forearms and hands. Clots form inside the inflamed vessels and block them. Unlike atherosclerosis, there is little fatty plaque. The disease usually starts at the fingertips and toes and moves upward. The body forms small collateral vessels around blockages that look like corkscrews on scans.

Causes and risk factors

The exact cause is unknown, but tobacco is central. Almost everyone with Buerger's disease uses tobacco: cigarettes, cigars, chewing tobacco, snuff, or heavy exposure to secondhand smoke. Cannabis smoking has also been linked. Tobacco is thought to trigger an immune reaction in people with a genetic predisposition.

Other risk factors:

• Age under 45 at onset
• Male sex, though rates in women have risen with smoking
• Chronic gum disease, possibly as an immune trigger
• Heavy tobacco use, including hand-rolled cigarettes

Symptoms

• Claudication: cramping pain in the arch of the foot, calf, hands or forearms with use, relieved by rest
• Rest pain: constant pain in the toes or fingers, often worse at night
• Cold, numb or tingling hands and feet
• Color changes: pale, bluish or red digits
• Raynaud's phenomenon: fingers or toes turning white then blue in the cold
• Painful ulcers on the tips of the toes or fingers
• Superficial thrombophlebitis: tender, red cords along veins
• Gangrene: dead, blackened tissue in advanced disease

Two or more limbs are usually affected, and hands are involved in many people, which helps distinguish it from typical artery disease.

Buerger's disease vs peripheral artery disease

Diagnosis

No single blood test confirms Buerger's disease. Doctors look for:

• tobacco use and onset before 45
• signs of blocked small arteries in the hands or feet
• absence of diabetes, high cholesterol, autoimmune disease, blood-clotting disorders or a source of clots from the heart

Tests include:

• Allen test: checks blood flow to the hand
• Ankle-brachial index and Doppler ultrasound
• Angiography (CT, MRI or catheter) showing blocked small vessels with corkscrew collaterals
• Blood tests to exclude vasculitis, lupus, scleroderma, clotting disorders and diabetes
• Echocardiogram to exclude clots from the heart

Treatment

Stopping tobacco completely

This is the only treatment proven to stop the disease. Even a few cigarettes a day, nicotine-containing products or chewing tobacco can keep it active. People who stop completely before gangrene develops very rarely need amputation. Nicotine replacement products may also aggravate the disease, so discuss options such as varenicline or bupropion and counseling with your doctor.

Other treatments

• Wound care for ulcers
• Pain control, which can be severe
• Medicines that widen blood vessels or improve blood flow, such as cilostazol or calcium channel blockers in some cases, and prostacyclin analogs such as iloprost in specialist centers
• Antibiotics for infected ulcers
• Protecting the hands and feet from cold, injury and tight shoes
• Walking exercise to encourage collateral circulation
• Sympathectomy (cutting nerves that narrow blood vessels) or spinal cord stimulation in selected people
• Bypass surgery is rarely possible because the affected vessels are small
• Amputation of dead tissue if gangrene develops

Outlook

If tobacco is stopped completely, the disease usually stabilizes and the risk of amputation falls sharply. People who keep using tobacco have a high risk of losing fingers, toes or limbs. Buerger's disease does not usually affect the heart or brain, and life expectancy is generally normal.

Frequently asked questions

What causes Buerger's disease?

The cause is not fully understood, but tobacco use is the key trigger. Almost everyone with Buerger's disease smokes or uses other tobacco products. It likely involves an immune reaction to tobacco components in genetically susceptible people.

Can Buerger's disease be cured?

There is no cure, but stopping all tobacco use halts the disease in most people. Ulcers often heal and pain improves after quitting. Continuing to smoke, even lightly, causes progression and a high risk of amputation.

What are the first signs of Buerger's disease?

Early signs include cramping pain in the feet or hands with use, cold or numb fingers and toes, color changes in the cold, and tender red cords along veins. Later, painful ulcers appear on the tips of the toes or fingers.

Can nonsmokers get Buerger's disease?

It is extremely rare in people who have never used tobacco. Most suspected cases in nonsmokers turn out to be other conditions, such as autoimmune vasculitis or clotting disorders, which need different treatment.