Buerger's disease

Buerger's disease is a rare disease of the arteries and veins in the arms and legs. In Buerger's disease — also called thromboangiitis obliterans — 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 Buerger's disease. For those who don't quit, amputation of all or part of a limb is sometimes necessary.

Symptoms

Buerger's disease symptoms include:

• Tingling or numbness in the hands or feet.
• Pale, reddish or blue-tinted hands or feet.
• 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.

When to see a doctor

See your doctor if you think you may have signs or symptoms of Buerger's disease.

Causes

The exact cause of Buerger's disease is unknown. While tobacco use clearly plays a role in the development of Buerger's disease, it's not clear how it does so. It's thought that chemicals in tobacco may irritate the lining of your blood vessels, causing them to swell.

Experts suspect that some people may have a genetic predisposition to the disease. It's also possible that the disease is caused by an autoimmune response in which the body's immune system mistakenly attacks healthy tissue.

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 and those who smoke more than a pack and half of cigarettes a day may have the greatest risk of Buerger's disease. 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 has been linked to the development of Buerger's disease, though the reason for this connection isn't yet clear.

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

Quit using tobacco in any form

Virtually everyone who has Buerger's disease has used tobacco in some form, most prominently cigarettes. To prevent Buerger's disease, it's important to not use tobacco.

Quitting smoking can be hard. If you're like most people who smoke, you've probably tried to quit in the past. It's never too late to try again. Talk to your doctor about strategies to help you quit.

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 autoimmune diseases such as scleroderma or lupus, blood-clotting disorders, and diabetes.

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 make any artery blockages easier to see 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.

Treatment

Smoking cessation

Although no treatment can cure Buerger's disease, the most effective way to stop the disease from getting worse 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.

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.

Other treatments

Other treatment approaches exist for Buerger's disease, but are less effective than quitting smoking. Options include:

• Medications to dilate blood vessels, improve blood flow or dissolve blood clots
• Intermittent compression of the arms and legs to increase blood flow to your extremities
• Spinal cord stimulation
• Amputation, if infection or gangrene occurs

Potential future treatments

• Nerve surgery. Surgery to cut the nerves to the affected area (surgical sympathectomy) to control pain and increase blood flow, although this procedure is controversial and long-term results haven't been well-studied
• Growing new blood vessels. Medications to stimulate growth of new blood vessels (therapeutic angiogenesis), an approach that is considered experimental
• Bosentan (Tracleer). This medication has been approved for treating high blood pressure in the lungs. The drug improved blood flow in small studies of people with Buerger's disease.
• Blood vessel procedure. A thin catheter threaded into the blood vessels might open blood vessels, restoring blood flow. Although this procedure — called endovascular therapy — isn't widely used, it might be effective.

Lifestyle and home remedies

There are a number of things you can do on your own to help improve your symptoms, such as:

• Exercise. Getting regular physical activity can benefit you in a number of ways, including helping to ease some of the pain of Buerger's disease. Try to get at least 30 minutes of moderate aerobic activity, such as walking or biking, most days of the week.
• Skin care. Take care of your fingers and toes if you have Buerger's disease. Check the skin on your arms and legs daily for cuts and scrapes, keeping in mind that if you've lost feeling to a finger or toe you may not feel, for example, a cut when it happens. Keep your fingers and toes protected and avoid exposing them to cold.

• Infection prevention. Low blood flow to your extremities means your body can't resist infection as easily. Small cuts and scrapes can easily turn into serious infections.

  Clean any cut with soap and water, apply antibiotic ointment, and cover it with a clean bandage. Keep an eye on any cuts or scrapes to make sure they're healing. If they get worse or heal slowly, see your doctor promptly.

• Gum care. Visit your dentist regularly to keep your gums and teeth in good health and avoid gum disease, which in its chronic form is linked to Buerger's disease.
• Avoiding other people's smoke. In addition to not smoking yourself, it's important to avoid secondhand smoke too.

Preparing for an appointment

You're likely to start by seeing your family doctor or primary care provider, who may eventually refer you to someone who specializes in blood vessel diseases (vascular specialist).

What you can do

To make the most of your appointment, come prepared with information and questions for your doctor.

• Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
• Write down key personal information, including whether you've ever smoked, and how many packs a day, or if you're exposed to trauma to your hands or feet, such as from using a jackhammer or other vibrating tools.
• Make a list of all medications, as well as any vitamins or supplements, that you're taking.
• Take a family member or friend along, if possible. Sometimes it can be difficult to remember all the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
• Write down questions to ask your doctor.

Some basic questions to ask your doctor include:

• What's the most likely cause of my symptoms?
• Are there other possible causes for my symptoms?
• What kinds of tests do I need?
• Is my condition likely temporary or long-lasting?
• What treatment options are available, and which do you recommend?
• I have these other health conditions. How can I best manage these conditions together?
• Are there any restrictions that I need to follow?
• Should I see a specialist?
• Are there any brochures or other printed material that I can take home with me? What websites do you recommend visiting?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions during your appointment.

What to expect from your doctor

Your doctor may ask:

• When did you begin experiencing symptoms?
• Do you have symptoms all the time, or do they come and go?
• How severe are your symptoms?
• Does anything seem to improve your symptoms?
• What, if anything, appears to worsen your symptoms?
• Do you use tobacco in any form now or have you ever used it?
• Do your fingers change color in response to cold?
• Have you had repetitive trauma to the affected area from tools?

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