Bürger's disease - frequency and age
Bürger’s disease is a very rare disease that occurs worldwide. In Europe, comparatively few people contract the disease. In contrast, the frequency is significantly higher in the populations of India, Korea and Japan, as well as among Ashkenazi Jews. Experts estimate that Bürger’s disease affects an average of 1 to 5 people in 10,000.
The disease primarily affects younger men between the ages of 17 and 44 who smoke heavily (cigarettes, cigarillos, cigars). In the past, medical professionals almost exclusively saw men suffering from Bürger’s disease during their consultations. This has changed in recent years – probably because women are also smoking more. Today, experts estimate that men are “only” affected about three times as often as women.
The cause of the disease is not yet fully understood, but the immune system is probably an important player. Smoking also appears to play a decisive role in the development of Bürger’s disease.
Any physical illness can also be associated with psychological stress. This can manifest itself in worry, tension, circling thoughts or sleep disorders, among other things, and make the course of treatment more difficult. If you or your relatives desire psychiatric-psychological counseling and support, our specialists at the USZ will be happy to assist you.
Bürger's disease: causes and risk factors
The causes of Bürger’s disease are still largely unclear. Researchers do not yet know exactly how the vascular disease develops. The following theories exist:
- A genetic predisposition may play a role in making people more susceptible to the disease.
- The immune system also appears to be an important player. Some researchers assume that this is an autoimmune disease in which the immune system mistakenly attacks the body’s own structures.
- The trigger for this immune system attack could be nicotine abuse. This is obvious because those affected are usually heavy smokers. It is possible that the ingredients in tobacco irritate the inner walls of the blood vessels and cause them to swell. People who roll their own cigarettes with raw tobacco and smoke more than one and a half packs a day have the highest risk of Bürger’s disease.
- Chronic gum inflammation is discussed as a risk factor, but the connection has not been scientifically proven.
It is known that the disease regresses if you stop smoking and abstain from nicotine. Anyone who smokes again, even just one cigarette, rekindles Bürger’s disease – a relapse occurs.
Symptoms: Bürger's disease often affects hands and feet
Bürger’s disease usually first manifests itself on the hands and feet. At an advanced stage, the vascular disease can affect larger areas of the arms and legs.
Bürger’s disease can cause various symptoms. These include, for example:
- Pain in the affected extremity: It can occur at rest, but also when you use your hands and feet or stop moving; the pain comes and goes.
- Bluish discoloration of the fingers and toes (cyanosis) due to reduced blood flow
- Phlebitis migrans/saltans: An inflammation of superficial veins that recurs repeatedly and can occur in different places.
- Symptoms of “PAD” (intermittent claudication or peripheral arterial occlusive disease, PAD for short): those affected have to stop more often when walking due to circulatory problems and pain in the legs. The symptoms appear in the area of the foot, the sole of the foot or the calf.
- Symptoms of Raynaud’s phenomenon: The extremities suddenly turn pale when exposed to cold or stress. Then they turn bluish and some time later reddish when the blood circulation starts again.
- Painful ulcers (gangrene) and dead tissue (necrosis) on the fingers and toes as well as a foul odor if the disease is advanced – amputation of the finger or toe is then often unavoidable.
- Paresthesia in the affected limbs (paresthesia), for example tingling, formication or numbness
- Water retention (edema)
- Feeling of coldness at the ends of the limbs due to poor circulation
Diagnosis of Bürger's disease
There is no specific test that we can use to diagnose Bürger’s disease. The diagnosis always begins with a medical history interview with the doctor. The following questions may indicate thrombangiitis obliterans:
- What exactly are your symptoms, how long have they been present and how severe are they?
- Where do you feel the discomfort: in your fingers, arms, toes, legs?
- Do you smoke? If yes: Since when and how much?
- How old are you?
- Do you have any known diseases?
This is usually followed by a physical examination. The extremities are examined for abnormalities such as bluish discoloration of the skin, swelling, superficial phlebitis or ulcers. Other diseases must be ruled out as the cause of the symptoms, such as
- Arteriosclerosis and peripheral arterial occlusive disease (PAD): The deposits in the vessels also cause constrictions and circulatory disorders – with the corresponding symptoms.
- Vascular inflammation (vasculitis)
- Arterial embolism (blockage of arteries by clots)
- Chronic venous insufficiency (venous insufficiency)
A blood test does not usually provide meaningful results that would be conclusive proof of a diagnosis of Bürger’s disease. For example, the inflammation values C-reactive protein (CRP) or the erythrocyte sedimentation rate (ESR) are usually normal despite the inflammation. However, other diseases can often be ruled out on the basis of the blood values, for example diabetes, coagulation disorders or rare autoimmune diseases such as scleroderma or lupus.
We use the following imaging procedures to diagnose Bürger’s disease:
- Color-coded duplex sonography: Ultrasound examination that can be used to visualize the blood vessels. The condition of the vessel wall, the course of the blood vessel and also the blood flow can be assessed. Constrictions or even occlusions of the arteries can be diagnosed.
- Magnetic resonance imaging (MRI) and magnetic resonance angiography: (MRA) MRI and MRA are a method of examining the vessels, the vessel wall and neighboring structures. In MRA, you are given a contrast agent via a vein and images are then taken. Constrictions, occlusions, dilatations and changes to the vessel wall can be detected.
- Tissue sampling (biopsy): A tissue sample is rarely necessary, but is the only way to make a definitive diagnosis. Patients with nodules in the skin or suspicious superficial phlebitis should have a biopsy. A tissue sample is taken and then examined under a microscope in the pathology department. This histological method can usually confirm the diagnosis of “Bürger’s disease”.
Bürger's disease: prevention, early detection, prognosis
The best way to prevent Bürger’s disease is not to start smoking in the first place. And if you are a smoker: Try a strict smoking cessation program! If you can’t do it on your own, we can offer you professional support. For example in our smoking cessation consultation. Stopping smoking can bring Bürger’s disease to a halt and improve the prognosis. If you continue to smoke, the prognosis is unfavorable.
There is no special option for the early detection of Bürger’s disease. Seek our help if you experience suspicious symptoms such as pain in the arms or legs, discomfort or skin discoloration.
Progression and prognosis of Bürger’s disease
Bürger’s disease is often acute and very severe. The circulatory disorders can be so severe that amputation is unavoidable within a short time. However, a chronic course is also possible with Bürger’s disease. In this case, the blood circulation in the extremities deteriorates slowly and gradually. Tissue that no longer receives sufficient oxygen and nutrients to survive dies. In this case, an amputation is necessary.
Treatment of Bürger's disease
The main treatment for Bürger’s disease is to strictly stop smoking. This is often difficult for those affected because they are unable to give up smoking. At the USZ we offer a specialized smoking cessation consultation. Incidentally, it is not enough to limit your tobacco consumption and still smoke a few cigarettes a day – the disease can get worse. Nicotine replacement products are also not advisable for quitting smoking in the case of Bürger’s disease because they contain nicotine, which can activate the vascular disease.