What is Buerger Disease?
Buerger disease, also known as thromboangiitis obliterans (TAO), is a disease that progressively inflames and blocks the blood flow in the small and medium arteries, typically in the arms and legs. It was first identified in 1879 by von Winiwarter, but the name “Buerger disease” came from Leo Buerger, who made significant observations of the disease in 1908 by studying the amputated limbs of affected patients. This disease mostly impacts males between the ages of 20 and 50 who smoke. In some cases, about 16%, it also causes inflammatory swelling in the superficial veins, indicating a body-wide inflammatory reaction.
Patients first experience pain and cramping (referred to as ‘claudication’) in the foot, leg, arm, or hand, which can sometimes be mistaken for a joint or muscle issue. As the disease progresses, claudication can also occur in the calf, eventually leading to rest pain, and sores or ulcers on the toes, feet, or fingers under rest conditions. This phenomenon is also known as Raynaud’s phenomenon.
The main treatment for Buerger disease is quitting smoking entirely. For patients who manage to quit smoking, the disease often dramatically improves, and the chance of avoiding limb amputation increases. Surgery doesn’t play a big role in treating this disease as there are often no suitable blood vessels for bypass surgery. Plus, the option to replace the affected blood vessel with a patient’s own vein is limited due to the presence of the inflammatory response in other veins.
What Causes Buerger Disease?
The exact cause of Buerger disease isn’t fully understood, yet it’s known that exposure to tobacco is necessary for the disease to both start and progress. We’re not sure how exactly the disease operates, but it might have to do with issues in the immune system and an overreaction to tobacco. This overreaction is related to increased sensitivity to certain types of collagen, a protein found in the body. Other symptoms include impaired blood vessel relaxation and higher levels of antibodies against the cells lining the blood vessels.
There could also be a genetic aspect to Buerger disease. People with the disease tend to have a higher occurrence of certain human leukocyte antigens (HLA) – proteins involved in the immune response – specifically HLA-A9, HLA-B5, and HLA-54.
Risk Factors and Frequency for Buerger Disease
Buerger’s disease has been becoming less common over the past five years, mainly due to fewer people smoking and stricter diagnostic criteria. People of Ashkenazi Jewish descent from Israel, as well as people of Indian, Korean, and Japanese ancestry are most commonly affected. This disease is less common in people of northern European heritage. Death from Buerger’s disease is uncommon, but the disease does cause significant health effects. If patients who are affected continue to smoke, 43% will require one or more amputations within approximately 7.6 years.
Looking at the history in the United States, Buerger’s disease was much more common in 1947 with 104 cases per 100,000 people. Nowadays, this has gone down to about 12.6 to 20 cases per 100,000 people.
- Buerger’s disease typically affects people between the ages of 20 and 45.
- It doesn’t happen in children or the elderly.
- The disease is more common in men, with a ratio of 3 men for every 1 woman. However, more women are getting the disease possibly due to an increase in smoking among women.
Signs and Symptoms of Buerger Disease
Buerger’s disease is a condition that leads to blockages in small to medium-sized veins and arteries. It’s accompanied by a strong response from white blood cells, tiny pockets of pus, and large multi-nucleated cells. As the disease progresses, the amount of hyperactively dividing cells decreases and the blood vessel often reopens. In the final stages of the disease, blood clots become established and the blood vessels become scarred.
While it’s common in Asia, it’s not common in males in North America, being diagnosed in less than 1% of patients with severe limb ischemia – a condition where not enough blood is getting to the limbs.
Buerger’s disease differs from atherosclerosis, a disease involving plaque build-up in the arteries. Instead, Buerger’s disease involves white blood cells spreading throughout all three layers of the artery wall. Some patients may develop an immune response against specific arterial components and have increased levels of immune complexes in circulation. Despite these possible signs, a firm diagnosis requires a tissue biopsy.
Buerger’s disease usually first affects the ends of the hands and feet, but as the disease worsens, it spreads to larger vessels closer to the body. The symptoms can range from pain in the arch of the foot and calf to pain in the hands, feet, and fingers even at rest. Nearly half of all cases may experience complications related to superficial vein inflammation. Also, nerve involvement may cause tingling or numbness in the fingertips and toes.
Testing for Buerger Disease
If doctors suspect you have Buerger disease, they won’t be able to confirm it with specific laboratory tests. However, they can rule out similar conditions by conducting comprehensive blood tests. These tests would check for conditions known to cause poor circulation in your fingers or toes like diabetes, immune system disorders, or abnormalities that make your blood clot too easily. In some cases, checking for a particular antibody in your blood (the anticardiolipin antibody) may provide insight into when the disease could start and whether you’re at risk for needing an amputation.
Another kind of test doctors often use in diagnosing Buerger Disease is an arteriography. This procedure allows them to see the flow of blood in your small- and medium-sized arteries, which can be affected by Buerger disease. With this test, they may see arteries show “pig-tailing” or “corkscrewing” which indicates unusual patterns of blood flow around blocked arteries. However, these signs don’t always mean that Buerger’s disease is present. They might also order an echocardiogram, which allows them to see images of your heart and rule out the possibility of clot formation originating from the heart.
Buerger disease affects your blood vessels over time and proceeds through different phases which can be examined under a microscope. Initially, inflammation leads to an infiltration of a type of white blood cell (neutrophils) and the formation of tiny nodules of inflammation (granulomas) that can block the blood vessels, while the walls of the vessels remain relatively unaffected.
Later on, the thrombus or blood clot becomes more organized as platelets continue to stick together. The final stage, which resembles other vessel diseases, reveals blood vessels blocked by an ‘organized’ clot and scarring tissue, with the inflammation no longer present. While this stage can look similar to other diseases that affect the blood vessels, Buerger’s disease can be distinguished by the fact that a specific part of the blood vessel – the internal elastic lamina – remains intact.
Treatment Options for Buerger Disease
Even though there is currently no cure for Buerger’s disease, the main way to manage the disease is to stop smoking entirely. Even light smoking or using nicotine replacement products, such as patches or gums, can continue to trigger the disease.
Medications like calcium channel blockers or other drugs that expand blood vessels can be used to alleviate Buerger’s disease symptoms, especially when coupled with a condition known as Raynaud’s disease. Doctors can also prescribe prostaglandin analogs, like intravenous iloprost, to help with pain and issues caused by poor blood flow to your limbs. While vascular endothelial growth factor (VEGF) injections are being tested for the treatment of Buerger’s disease, the effectiveness of this approach remains inconclusive. Treatments like lumbar and/or cervical sympathectomy, or the use of spinal cord stimulators, have occasionally been used.
Intravenous iloprost can help reduce symptoms, improve the health of the skin and tissues in the distal extremities (which are farthest from the center of the body), and lower the rate of amputations resulting from Buerger’s disease. This treatment is particularly effective at slowing the loss of tissue and reducing the need for amputation in patients who quit smoking and have severely limited blood flow to the limbs.
Pain resulting from poor blood flow can be alleviated with non-steroidal anti-inflammatory drugs (NSAIDs) and narcotic pain relief medications. Mild distal extremity ulcers can be treated with appropriate antibiotics.
Hyperbaric oxygen therapy, where a patient inhales pure oxygen in a pressurized room or a tube, can be used to enhance healing in patients with conditions like diabetic wounds, bone infections that don’t respond to treatment, restricted blood flow to limbs, or fleshy tissue infections. Although there’s limited data on its effectiveness for Buerger’s disease patients without available revascularization treatments, its usage remains experimental for such cases.
Because Buerger’s disease often affects small and medium-sized arteries throughout the body, a surgical procedure to restore normal blood flow is usually not recommended. If a patient continues to smoke, the final surgical treatment could be to amputate a lower limb which has non-healing ulcers, gangrene, or constant pain. However, amputation should be avoided if at all possible.
What else can Buerger Disease be?
When trying to diagnose Buerger disease, doctors need to think about a variety of conditions that might cause similar symptoms. Some of these include:
- Antiphospholipid syndrome (a disorder of the immune system that increases the risk of blood clots) and pregnancy
- Atherosclerosis (hardening of the arteries)
- Frostbite (damage to skin and tissue caused by exposure to freezing temperatures)
- Giant cell arteritis (an inflammation of the lining of your arteries)
- Gout (a form of arthritis characterized by severe pain and redness in the joints)
- Imaging findings in polyarteritis nodosa (a serious blood vessel disease)
- Raynaud’s syndrome (a rare disorder of the blood vessels usually in fingers and toes)
- Scleroderma (a group of rare diseases that involve the hardening and tightening of the skin and connective tissues)
- Systemic lupus erythematosus (an autoimmune disease in which the body’s immune system attacks its own tissues and organs)
- Type 1 diabetes (a chronic condition where the pancreas produces little or no insulin)
- Type 2 diabetes (a chronic condition that affects the way the body processes blood sugar)
Each of these conditions needs to be considered and ruled out by conducting appropriate tests in order to make an accurate diagnosis.
What to expect with Buerger Disease
Dying from Buerger disease is uncommon. From 1999 to 2007, the U.S Centers for Disease Control and Prevention (CDC) recorded 117 deaths in the United States caused by Buerger disease.
There’s a clear difference in the outcomes for patients with Buerger disease, which hinge on whether they completely stop using tobacco or not.
Possible Complications When Diagnosed with Buerger Disease
Buerger’s disease can result in several potential complications such as:
- Gangrene
- Ulcerations
- Infection
- Amputation
- Blockage of arteries in the heart, spleen, kidneys, or intestines (although quite rarely)
Preventing Buerger Disease
People dealing with Buerger’s disease need to be strongly urged to stop using all forms of tobacco. They need to know that if they can give up smoking, their disease may start to get better and they may be able to avoid amputation.
Healthcare professionals should also be clear that for their disease to improve, patients must seriously minimize their exposure to secondhand smoke. This can be particularly tough for people who live with a smoker. So, referring these patients and their smoking companions to combined efforts to quit smoking might be a good option.
