Buerger's Disease: Not from Eating Hamburgers

Janet Smith came to our clinic with a sore on her left foot that after several months had not healed. Before she had the sore, Janet remembered feeling pain in the instep of her foot when walking. Her pain had become steadily worse, and eventually became more constant, occurring at night while lying in bed as well as when walking. Pain in the leg or foot when exercising indicated that she had intermittent claudication, a symptom that is common in persons with peripheral arterial disease (PAD). The development of pain at rest and sores were indicative of critical limb ischemia (CLI) (see page 2), which indicated that her vascular disease was quite advanced. Yet some aspects of her case were not typical for PAD. The history she told was remarkable because six years ago, at age 28, she had a gangrenous right second toe requiring peripheral bypass surgery and amputation. PAD is very uncommon in anyone less than 40 years of age, particularly in women. Unlike most patients with PAD, Janet did not have a history of high cholesterol, high blood pressure, diabetes, or other risk factors that are associated with artery blockage from plaque. However, she did have one risk factor that had increased her risk for arterial disease. She had smoked a pack of cigarettes each day for the past 18 years, starting when she was 16.

Janet's story is a typical one for Buerger's disease or Thromboangiitis obliterans (TAO). Thromboangiitis obliterans is a rare disorder characterized by inflammation of the small and medium arteries and veins. It affects about 8-11 per 100,000 of North Americans. The inflammation in TAO frequently leads to blockages of arteries of the lower segments of the arms and legs, and may cause claudication or rest pain and non-healing sores or ulcers (CLI). TAO occurs exclusively in individuals with a history of tobacco exposure of any kind, including smoking, chewing or snuff. This suggests that tobacco is an essential cause of TAO.

TAO is different from PAD, because it is not caused by atherosclerosis (plaque) buildup that causes a narrowing of the artery. Instead TAO is caused by inflammation of the artery wall, along with the development of clots in the small and medium sized arteries of the arms or legs causing the arteries to become blocked. Without blood flow below the inflamed artery or clots, the fingers, toes, and skin tissue do not receive adequate blood. This usually leads to enormous pain at rest or with exercise, sores may develop and may be slow to heal.

There are four key factors physicians use to diagnose TAO: (1) Rest pain or ulceration before 50 years of age, (2) tobacco use, and (3) tests indicating the arteries are blocked. Typical tests include artery blood flow measurements (such as the ABI, or other vascular laboratory tests, such as ultrasound), arteriography (pictures of the affected blood vessel obtained by injecting a dye via a catheter), and/or biopsy of the affected artery. As well, doctors would usually want to (4) exclude other causes for artery blockage or clot development. A physician would want to be certain that a clot did not develop from the heart or a large blood vessel and travel to the arm or leg (an embolus), that there had been no blood vessel injury or trauma, no local lesions such as a blood vessel cyst, no autoimmune diseases such as scleroderma, and no blood clotting diseases.

Unfortunately, knowledge about TAO is limited, and the long-term (greater than 15 year) risk of amputation and death is not well known. One widely cited study of 112 patients was gathered from the Cleveland Clinic Foundation from 1970 to 1987. The study revealed that skin ulcerations occurred among 76% of TAO patients. Additionally, 27% of TAO patients underwent one or more amputations (15% finger, 33% toe, 10% forefoot, 36% below the knee, 5% above the knee). Clots in the superficial veins of the arms and legs and Raynaud's phenomenon (fingers turning white and painful upon cold exposure) are also common. Despite these statistics, there is also good news. TAO almost never affects the arteries to the brain or heart, and thus life expectancy among persons with TAO is reported to be normal.

The treatment for TAO is immediate and complete tobacco cessation. Mayo Clinic physicians determined that TAO patients who continue to smoke have a high rate of amputation that persists up to 17 years after first diagnosis. The risk of amputation in TAO patients who stop smoking is much lower.

Janet had good fortune and a strong will. She stopped smoking and with aggressive wound care and the use of special shoes she was able to heal her sores and avoid another amputation. Visit www.vdf.org for links to more information about TAO.

About the author: Dr. Leslie T. Cooper is a vascular specialist with the Department of Cardiovascular Diseases of the Mayo Clinic in Rochester, Minnesota.

Note: The name of the patient and specific details of her care were changed to protect her privacy.