Buerger’s disease

Synonyms

5

Overview

Buerger's disease (thromboangiitis obliterans) is a rare disease of the arteries and veins in the arms and legs. In Buerger's disease, your blood vessels become inflamed, swell and can become blocked with blood clots (thrombi).

This eventually damages or destroys skin tissues and may lead to infection and gangrene. Buerger's disease usually first shows in your hands and feet and may eventually affect larger areas of your arms and legs.

Virtually everyone diagnosed with Buerger's disease smokes cigarettes or uses other forms of tobacco, such as chewing tobacco. Quitting all forms of tobacco is the only way to stop . For those who don't quit, amputation of all or part of a limb is sometimes necessary.

Symptoms

Buerger's disease symptoms include:

  • Pain that may come and go in your legs and feet or in your arms and hands. This pain may occur when you use your hands or feet and eases when you stop that activity (claudication), or when you're at rest
  • Inflammation along a vein just below the skin's surface (due to a blood clot in the vein)
  • Fingers and toes that turn pale when exposed to cold (Raynaud's phenomenon)
  • Painful open sores on your fingers and toes
  • Arterial thrombosis
  • Gangrene
  • Skin ulcer
  • Vasculitis

Causes

The exact cause of Buerger's disease is unknown. Buerger disease has a strong relationship to cigarette smoking. This association may be due to direct poisioning 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's also possible that the disease is caused by an autoimmune response in which the body's immune system mistakenly attacks healthy tissue. It is possible that these genetic influences account for the higher prevalence of Buerger disease in people of Israeli, Indian subcontinent, and Japanese descent. Certain HLA (human leukocyte antigen) haplotypes have also been found in association with Buerger disease.

 It is seen most often in young to middle-aged men (ages 20-40) who are heavy smokers of cigarettes. Cases of this disease in non-smokers are very rare, hence, cigarette smoking is considered a causative factor. Approximately 40% of the patients have a history of inflammation of a vein (phlebitis), which may play a role in the development of Buerger's disease. The disease is mainly seen in the legs of affected persons, but may also appear in their arms.

Risk factors:

  • Tobacco use: Cigarette smoking greatly increases your risk of Buerger's disease. But Buerger's disease can occur in people who use any form of tobacco, including cigars and chewing tobacco. People who smoke hand-rolled cigarettes using raw tobacco may have the greatest risk of Buerger's disease. It isn't clear how tobacco use increases your risk of Buerger's disease, but virtually everyone diagnosed with Buerger's disease uses tobacco. It's thought that chemicals in tobacco may irritate the lining of your blood vessels, causing them to swell. The rates of Buerger's disease are highest in areas of the Mediterranean, Middle East and Asia where heavy smoking is most common.
  • Chronic gum disease: Long-term infection of the gums also is linked to the development of Buerger's disease.
  • Sex: Buerger's disease is far more common in males than in females. However, this difference may be linked to higher rates of smoking in men.
  • Age: The disease often first appears in people less than 45 years old.

Complications:

If Buerger's disease worsens, blood flow to your arms and legs decreases. This is due to blockages that make it hard for blood to reach the tips of your fingers and toes. Tissues that don't receive blood don't get the oxygen and nutrients they need to survive.

This can cause the skin and tissue on the ends of your fingers and toes to die (gangrene). Signs and symptoms of gangrene include black or blue skin, a loss of feeling in the affected finger or toe, and a foul smell from the affected area. Gangrene is a serious condition that usually requires amputation of the affected finger or toe.

Prevention

The cause of the disease is thought to be autoimmune in nature and heavily linked to tobacco use in patients with Buerger's as primary disease.

Diagnosis

While no tests can confirm whether you have Buerger's disease, your doctor will likely order tests to rule out other more common conditions or confirm suspicion of Buerger's disease brought on by your signs and symptoms. Tests may include:

Blood tests:

Blood tests to look for certain substances can rule out other conditions that may cause similar signs and symptoms. For instance, blood tests can help rule out scleroderma, lupus, blood-clotting disorders and diabetes, along with other diseases and conditions.

The Allen's test:

Your doctor may perform a simple test called the Allen's test to check blood flow through the arteries carrying blood to your hands. In the Allen's test, you make a tight fist, which forces the blood out of your hand. Your doctor presses on the arteries at each side of your wrist to slow the flow of blood back into your hand, making your hand lose its normal color.

Next, you open your hand and your doctor releases the pressure on one artery, then the other. How quickly the color returns to your hand may give a general indication about the health of your arteries. Slow blood flow into your hand may indicate a problem, such as Buerger's disease.

Angiogram:

An angiogram helps to see the condition of your arteries. An angiogram can be done non-invasively with the use of CT or MRI scans.

Or it may be done by threading a catheter into an artery. During this procedure, a special dye is injected into the artery, after which you undergo a series of rapid X-rays. The dye helps to delineate any artery blockages that show up on the images.

Your doctor may order angiograms of both your arms and your legs — even if you don't have signs and symptoms of Buerger's disease in all of your limbs. Buerger's disease almost always affects more than one limb, so even though you may not have signs and symptoms in your other limbs, this test may detect early signs of vessel damage.

Prognosis

Buerger's is not immediately fatal. Amputation is common and major amputations (of limbs rather than fingers/toes) are almost twice as common in patients who continue to smoke. Prognosis markedly improves if a person quits smoking. Female patients tend to show much higher longevity rates than men, as is in society. The only way to slow the progression of the disease is to abstain from all tobacco products.

Treatment

Smoking cessation:
Although no treatment can cure Buerger's disease, the most effective way to halt the disease's progress is to quit using all tobacco products. Even a few cigarettes a day can worsen the disease.
Your doctor can counsel you and recommend medications to help you stop smoking and stop the swelling in your blood vessels. You'll need to avoid nicotine replacement products because they supply nicotine, which activates Buerger's disease; there are non-nicotine products that you can use. If the disease is still active, your doctor may check your urine for the presence of nicotine to see if you're still smoking.
Another option is a residential smoking cessation program. In these programs, you stay at a treatment facility, sometimes a hospital, for a set number of days or weeks. During that time, you participate in daily counseling sessions and other activities to help you deal with the cravings for cigarettes and to help you learn to live tobacco-free. Continued smoking is associated with an overall amputation rate of 40 to 50 percent.

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) , although this procedure is controversial and long-term results haven't been well-studied
  • Therapeutic angiogenesis (medications to stimulate growth of new blood vessels) an approach that is considered experimental
  • Spinal cord stimulation
  • Amputation, if infection or gangrene occurs
  • Intermittent compression of the arms and legs to increase blood flow to your extremities

Resources

  • NIH
  • Mayo Clinic