Buerger's disease causes inflammation of blood vessels in legs and arms, particularly in hands and feet. It leads to narrowing and blockage of the blood vessels so that blood flow to hands and feet is reduced. This causes pain and other symptoms and may eventually lead to damage and death of the tissues in hands and/or feet. The exact cause is not known but it affects smokers. The single most important thing that you can do if you are diagnosed with Buerger's disease is to stop smoking. This can help to stop the disease from getting worse and reduces your chance of needing amputation of a finger, a toe, or worse.
Buerger's disease is a condition that causes inflammation of the small and medium-sized blood vessels in legs and arms, particularly those in the hands and feet. It is usually arteries that are affected but it can sometimes affect veins.
There is inflammation of segments (sections) of the blood vessels. This inflammation is called vasculitis and it causes narrowing of the blood vessels. It can also cause the formation of small blood clots which can lead to complete blockage of the blood vessels. Because of the narrowed and blocked segments of the blood vessels, blood cannot pass through to all parts of the hands and feet as normal. This causes pain and other symptoms and may eventually lead to damage and death of the tissues in hands and/or feet.
The inflammation can skip segments of the blood vessels so that there may be normal segments in between the inflamed segments. Buerger's disease was described by Dr Leo Buerger in 1908. It is also called thromboangiitis obliterans.
The exact cause is not known. However, what is known is that the disease occurs in smokers. Smoking acts as some kind of a trigger and causes Buerger's disease to start. Continued smoking also causes the condition to progress (get worse). Occasionally, chewing tobacco has been shown to cause the disease. It has also occurred in people who smoke cannabis long-term.
It is thought that the immune system is involved in the development of Buerger's disease in some way. Buerger's disease might actually be an autoimmune condition that is triggered by nicotine. (Normally, our body makes antibodies to fight infections; for example, when we catch a cold or have a sore throat. These antibodies help to kill the germs causing the infection. In autoimmune diseases the body makes similar antibodies (autoantibodies) that attack its normal cells.)
Buerger's disease is rare in people who come from Northern Europe. It is more common in people of oriental race, including people from South-east Asia, India and the Middle East. Buerger's disease seems to be particularly common in people from Bangladesh who smoke a type of homemade cigarette made from raw tobacco.
Most people with Buerger's disease develop the condition between the ages of 20 and 45. It is more common in men. However, more women are being diagnosed with it as the number of women who smoke increases. Overall, the number of people who develop Buerger's disease is reducing as the number of people who smoke is decreasing.
As mentioned above, symptoms occur because blood is not able to get through to all parts of the hands and/or feet.
Your hands and feet may feel cold and they may appear slightly swollen. They may be pale, or have a red or bluish colour. Pain in your hands and feet is one of the main symptoms. This can sometimes feel like burning. You can develop pain in your legs or feet when you walk. This is called intermittent claudication. As the disease gets worse, pain can occur even when you are resting. Pain is often worse at night time. You may notice tingling or numbness in your feet and hands. Cold weather will usually make symptoms worse.
As Buerger's disease worsens, you may start to develop ulcers on your hands and feet, which can be painful. Skin infections can also set in. Gangrene can develop if the tissues in your hands and feet completely die due to lack of blood supply. If you develop gangrene, your skin and underlying tissues become black.
Out of your two feet and two hands, it is usually at least three of these that are affected in Buerger's disease. However, you may notice symptoms in just one finger of your hand or one toe on your foot.
Buerger's disease can sometimes be difficult to diagnose as there is no specific test that can confirm it. Therefore, it is important that doctors rule out any other conditions that could be causing your symptoms. For example, autoimmune diseases, blood clotting problems, diabetes, peripheral vascular disease caused by atheroma (see separate leaflet called 'Peripheral Arterial Disease in Legs' for further details), a blood clot that has travelled from elsewhere in your body such as your heart, etc.
A doctor will usually ask to examine your arms, hands, legs and feet. They may examine your pulses in your arms, legs and feet, which will usually be absent or reduced in Buerger's disease. They may suggest some blood tests to look for signs of other conditions such as those listed above.
A doctor may also suggest a test called angiography. For this test a special dye called contrast material is injected and then X-rays are used to show up the arteries in your arms, hands, legs and feet. Segments of narrowed and/or blocked arteries can be seen in the distal blood vessels (those closest to your hands and feet) if you have Buerger's disease. However, the proximal blood vessels (the ones closest to your body) will be normal. But, these changes can also occur in some other conditions, so again cannot give conclusive evidence of Buerger's disease. A biopsy (a sample of tissue) from one of the blood vessels may sometimes be taken to look for changes that may help to support the diagnosis.
Sometimes another test called a Doppler ultrasound scan is suggested. This uses ultrasound to look at the blood flow in the blood vessels of your arms and legs. Blood pressure cuffs may be put on and inflated at points along your arms or your legs during the test. Sometimes other tests may be advised such as an echocardiography (an ultrasound scan of your heart). This can help to exclude another source for the blood clots in your arms or your legs.
Because Buerger's disease can be difficult to diagnose, scoring systems have been developed to help doctors. Points are awarded depending on things such as whether you smoke, your age, your symptoms, any history of other medical problems, the findings at angiography, etc. Using these scoring systems, the likelihood of Buerger's disease can then be worked out. Essentially, the more points that you score, the more likely the diagnosis.
There is no cure for Buerger's disease. So, the aim of treatment is to stop the disease from progressing (getting any worse) and to control any symptoms that you may have. The following may be helpful.
The single most important thing that you can do if you are diagnosed with Buerger's disease is to stop smoking. Even smoking one cigarette a day can keep Buerger's disease active and allow it to progress and your symptoms to get worse. Stopping smoking is the only treatment known to be effective. Buerger's disease can become inactive when you quit smoking. There is also some evidence from studies that suggests that you should avoid secondhand smoke (passive smoking). For further information and help to quit smoking, please refer to the separate leaflet called 'Smoking - Tips to Help you Stop'.
If you have Buerger's disease, any infection of the skin of your hands and feet needs to be treated promptly with antibiotics. For example, infection of skin ulcers or infection of the skin after a minor injury. Painkilling drugs including non-steroidal anti-inflammatory drugs, or sometimes stronger drugs such as drugs containing codeine, may be needed.
It is not usually possible to open up blocked or narrowed blood vessels using surgical procedures in someone with Buerger's disease. However, sometimes surgical treatment may be needed if someone has a lot of tissue damage, or tissue death. For example, if someone has ulcers that are not healing, if gangrene has set in, or if someone has intractable pain. This is usually someone who continues to smoke despite the diagnosis.
Amputation is used to remove the damaged or dead tissue. Some people with Buerger's disease need to have a number of fingers or toes amputated. Some people need to have higher amputations (for example a whole foot, or their leg below the knee).
Surgery may also be used in some people for pain control. Cutting the nerves to the area affected may help to control pain. This is called a surgical sympathectomy.
There are some other treatments for Buerger's disease that have been used in research and trials and may not be widely available. They include treatment with a drug called iloprost. This is given intravenously (into your veins). It helps to relax the walls of blood vessels and so improve blood flow. It is also thought to reduce blood clotting. In some people with Buerger's disease who have just stopped smoking and who have severely reduced blood flow to their hands and/or feet, iloprost has been shown to improve symptoms, slow down the progression of the disease and also reduce the chance of needing an amputation.
Other research is looking at using 'clot busting' drugs and also gene therapy.
There are various other things that you can do if you are diagnosed with Buerger's disease. They include the following:
If you have Buerger's disease and you continue to smoke, you will increase your risk of needing to have one or more of your fingers or toes amputated because of ulceration or gangrene. Around 43 out of 100 people with Buerger's disease who continue to smoke will need at least one amputation over a 7- to 8-year period. This can be contrasted with the fact that among people with Buerger's disease who quit smoking, 94 out of 100 will avoid amputation.