Buerger disease

Other Names: Buerger's disease; Thromboangiitis obliterans; TAO; Inflammatory occlusive peripheral vascular disease; Occlusive peripheral vascular disease

Buerger disease is a disease in which small and medium-sized blood vessels in the arms and/or legs become inflamed and blocked (vasculitis). This reduces blood flow to affected areas of the body, eventually resulting damage to tissues. Average age when symptoms begin is around 35. Women and older adults are affected less often.

Cause

Buerger disease has a strong relationship to cigarette smoking. This association may be due to direct poisoning of cells from some component of tobacco, or by hypersensitivity to the same components. Many people with Buerger disease will show hypersensitivities to injection of tobacco extracts into their skin. There may be a genetic component to susceptibility to Buerger disease as well. It is possible that these genetic influences account for the higher prevalence of Buerger disease in people of Israeli, Indian subcontinent, and Japanese descent. It is also possible that Buerger disease is an autoimmune disease, as the immune system seems to play a large role in its development. More research is needed to identify the exact underlying causes

Riskfactors

This condition mostly affects young men ages 20 to 45 who are heavy smokers or chew tobacco. Female smokers may also be affected. The condition affects more people in the Middle East, Asia, the Mediterranean, and Eastern Europe. Many people with this problem have poor dental health, most likely due to tobacco use.

Symptoms

Symptoms most often affect 2 or more limbs and may include:

• Fingers or toes that appear pale, red, or bluish and feel cold to the touch.
• Sudden severe pain in the hands and feet. The pain may feel like a burning or tingling.
• Pain in the hands and feet that most often occurs when at rest. The pain may be worse when the hands and feet get cold or during emotional stress.
• Pain in the legs, ankles, or feet when walking (intermittent claudication). The pain is often located in the arch of the foot.
• Skin changes or small painful ulcers on the fingers or toes.
• Occasionally, arthritis in the wrists or knees develops before the blood vessels become blocked.

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.

80%-99% of people have these symptoms

• Arterial thrombosis(Blood clot in artery)
• Gangrene(Death of body tissue due to lack of blood flow or infection)
• Skin ulcer(Open skin sore)
• Vasculitis(Inflammation of blood vessel)

30%-79% of people have these symptoms

• Acrocyanosis(Persistent blue color of hands, feet, or parts of face)
• Arthralgia(Joint pain)
• Paresthesia(Pins and needles feeling)

5%-29% of people have these symptoms

• Hyperhidrosis(Excessive sweating)
• Insomnia(Difficulty staying or falling asleep)

Diagnosis

The following tests may show blockage of blood vessels in the affected hands or feet:

• Ultrasound of the blood vessels in the extremity, called plethysmography
• Doppler ultrasound of the extremity
• Catheter-based x-ray arteriogram
• Blood tests for other causes of inflamed blood vessels (vasculitis) and blocked (occlusion of) blood vessels may be done. These causes include diabetes, scleroderma, vasculitis, hypercoagulability, and atherosclerosis. There are no blood tests that diagnose thromboangiitis obliterans.

• A heart echocardiogram may be done to look for sources of blood clots. In rare cases when the diagnosis is unclear, a biopsy of the blood vessel is done.

Treatment

Currently there is not a cure for Buerger disease, however there are treatments that can help control it. The most essential part of treatment is to avoid all tobacco and nicotine products. Even one cigarette a day can worsen the disease.

The following treatments may also be helpful, but do not replace smoking/nicotine cessation:

• Medications to dilate blood vessels and improve blood flow (e.g., intravenous Iloprost)
• Medications to dissolve blood clots
• Treatment with calcium channel blockers
• Walking exercises
• Intermittent compression of the arms and legs to increase blood flow to your extremities
• Surgical sympathectomy (a controversial surgery to cut the nerves to the affected area to control pain and increase blood flow)
• Therapeutic angiogenesis (medications to stimulate growth of new blood vessels)
• Spinal cord stimulation
• Amputation, if infection or gangrene occurs
• Aspirin and medicines that open the blood vessels (vasodilators) may help. In very bad cases, surgery to cut the nerves to the area (surgical sympathectomy) can help control pain. Rarely, bypass surgery is considered in certain people.
• It may become necessary to amputate the fingers or toes if the area becomes very infected and tissue dies.

Prognosis

Symptoms of thromboangiitis obliterans may go away if the person stops tobacco use. People who continue to use tobacco may need repeated amputations.

Prevention

People with a history of Raynaud phenomenon or blue, painful fingers or toes, especially with ulcers, should not use any form of tobacco.

Epidemiology

Buerger disease has become less common over the past ten years given the decrease in smoking prevalence and more strict diagnostic criteria. In 1947, it was estimated to occur in 104 out of 100,000 people. It now is estimated to occur in 12-20 out of 100,000 people. This condition mostly affects young men ages 20 to 45 who are heavy smokers or chew tobacco. Female smokers may also be affected. The condition affects more people in the Middle East, Asia, the Mediterranean, and Eastern Europe. Many people with this problem have poor dental health, most likely due to tobacco use.

NIH genetic and rare disease info

• NIH info on Buerger disease

Buerger disease is a rare disease.

Buerger disease Resources
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