Buerger's disease (thromboangiitis obliterans): causes, symptoms, treatment

What is Buerger's disease?

Burger's diseaseThromboangiitis Obliterans (Thromboangiitis Obliterans) is a rare disease that in most cases affects young men and middle-aged men who smoke cigarettes. Thromboangiitis obliterans is characterized by narrowing or blockage (occlusion) of the veins and arteries in the extremities, which reduces blood flow to these areas (peripheral vascular disease). The legs are more often affected than the hands.

In most cases, the first symptom is severe pain in the lower arms and legs at rest. Sufferers experience leg cramps when walking, which in rare cases can cause lameness. Additionally, affected people may have sores (ulcers) on the limbs, numbness and tingling sensations, and also lack of normal blood flow to fingers and / or toes when exposed to low temperatures (Raynaud's syndrome), and / or inflammation and blockage of certain veins (thrombophlebitis).

In severe cases, in people with thromboangiitis obliterans, tissue death (gangrene) of the affected limbs is observed. The exact cause of Buerger's disease is not known; however, the majority of patients are young and adult male smokers.

Signs and symptoms

Burger's disease is characterized by narrowing or blockage (occlusion) of the intermediate and / or small-sized arteries and the veins of the extremities, which leads to a decrease in blood flow to these areas (peripheral vascular disease). The disease tends to come on suddenly (acutely), lasting 1-4 weeks. The disorder is recurrent.

In most cases, the first sign of Bürger's disease is severe pain in the lower arms and / or legs at rest. People may also experience leg cramps when walking, which in rare cases can lead to lameness.

Other physical signs associated with Buerger's disease may include:

• manifestations (ulcers) on the arms and legs;
• pallor of hands;
• numbness and tingling and lack of normal blood flow in the fingers and toes when exposed to low temperatures (Raynaud's syndrome);
• inflammation and blockage of certain veins (thrombophlebitis).

Dry, dark ulcerations that often form on the tips of the fingers or toes can be extremely painful. The pain associated with these ulcers may increase with elevation. In severe cases, patients with the disease may have tissue necrosis (gangrene) in the affected area.

In some cases, the arteries and veins of the intestine can also be affected. This can lead to extreme severity or abdominal pain and weight loss. In extremely rare cases (i.e. less than 2%), neurological disorders were identified in the affected individuals.

Causes and risk factors

The exact cause of Buerger's disease is unknown. However, the main factor causing the disorder is tobacco smoking.

In fact, most scientists assume that past or present tobacco use is a prerequisite for diagnosing Buerger's disease. The exact link between tobacco products and Buerger's disease is not fully understood.

Some scientists suggest that Buerger's disease may be autoimmune disease. In some cases, injuries to the arms and legs contribute to the disorder. Autoimmune disorders occur when the body's natural defenses against "foreign" or invading organisms (e.g. antibodies), i.e. the immune system begins to attack healthy tissues for unknown reasons reasons.

Genetic factors play a role in the development and severity of the disease, as prevalence varies greatly between ethical groups. More research is needed to determine the exact role that genetics plays in the development of the disease.

Symptoms of Buerger's disease are caused by impaired circulation (ischemia) in certain areas of the body, most often in the arms and legs.

Affected populations

Buerger's disease is a very rare disease that mostly affects young or middle-aged smokers, with symptoms onset before the age of 40-45. In recent years, more female victims have been reported in the medical literature. Some scientists suggest that this is due to an increase in the number of women who smoke. Once the ratio of affected men to women was 100: 1, recent articles in the medical literature suggest that the ratio could be 10: 1 or more. In very rare cases, the disease has been observed in non-smokers.

Burger's disease is extremely rare in Europe, but more common in other parts of the world, especially in parts of Asia and the Far and Middle East. The estimated incidence ranges from 12.6-20 per 100,000 people in the general population. The disease is more common in countries with heavy tobacco use.

Although the majority (majority) of cases are associated with tobacco smoking, the disorder is also has been observed in people who did not smoke but used smokeless tobacco (for example, chewing tobacco).

Diagnostics

The diagnosis of Buerger's disease is made by identifying characteristic physical signs and symptoms. Many doctors need a history of recent or current tobacco use when diagnosing Bürger's disease. To confirm the diagnosis, a study such as angiography or non-invasive methods is performed. During angiography, an injection of a specialized dye is used to show the blood vessels on x-rays.

Thromboangiitis obliterans can be mimicked by a variety of other conditions that reduce blood flow to the extremities. These other disorders should be ruled out by aggressive assessment, as their treatment differs significantly from the treatment for Buerger's disease.

Symptomatic Disorders

Symptoms of the following disorders may be similar to those of Buerger's disease. Comparisons can be useful for differential diagnosis:

• Vasculitis - inflammation of the blood vessels. In people with vasculitis, inflammation damages the lining of the affected blood vessels, causing narrowing, blood clots (thrombosis), and / or blockage. As a result, the blood supply of oxygen to certain tissues may be restricted (ischemia), which may lead to pain, tissue damage, and in some cases, disruption of the work of some of the affected organs. Vasculitis affects veins and arteries of any type or size; may affect one or more organs and tissues of the body; and be a primary (stand-alone) disease or arise from or in association with a number of different underlying disorders. Consequently, the range and severity of symptoms and signs associated with vasculitis varies greatly. The exact cause of vasculitis is not fully understood. However, it is believed that in most cases the disease is associated with disorders of the body's immune system.
• Scleroderma Is a rare connective tissue disorder characterized by abnormal thickening of the skin. Connective tissue is made up of collagen, which supports and binds other tissues in the body. There are several types of scleroderma. Some types affect specific parts of the body, while other types affect the entire body and internal organs (systemic). The exact cause of scleroderma is unknown. It is known that the immune system and the vascular system, as well as the metabolism of the connective tissue, play a role in the disease process.
• Takayasu's arteritis - a rare disease characterized by inflammation of the large elastic arteries. The main artery of the heart and the pulmonary artery are affected. The disorder causes progressive inflammation of many arteries in the body (polyarteritis), which results in decreased blood flow. Arteries in the head and arms can be affected, which can lead to the loss of key pulse points in the body. Some people with this arteritis have irregular narrowing of parts of large arteries. (segmental stenosis) and abnormal backflow of blood from the aorta to the left ventricle of the heart (aortic regurgitation).

Treatment

Treatment for Buerger's disease is symptomatic and supportive. Symptoms usually improve if people stop smoking. In some cases, when people quit smoking, complete remission of the disorder may occur.

If the sick person does not quit smoking, premature or unnecessary surgeries should be avoided when treating Buerger's disease. Conservative therapy may include the use of drugs that prevent blood clotting (anticoagulants), drugs that increase the diameter of blood blood vessels (vasodilators), drugs that prevent inflammation (anti-inflammatory drugs), antibiotics and / or drugs that reduce pain (analgesics).

In some cases, surgery is necessary. Affected people undergo surgery that destroys nerve endings (ganglia), interrupting the nerve pathway and improving the blood supply to the affected limb (sympathectomy). In some cases, surgeons may try bypass procedures to bypass blocked or narrow (occluded) veins and / or arteries. In severe cases, surgeons may be forced to remove (amputate) a toe or leg, or part of an arm or leg.

Forecast and prevention

If you stop using tobacco products, the symptoms associated with thromboangiitis obliterans may simply disappear without the need for treatment.

Thromboangiitis obliterans is not immediately fatal. Amputation is common, and major amputations are nearly twice as common in patients who continue to smoke. The prognosis will improve markedly if the patient quits smoking. Female patients tend to have much higher life expectancy than men. The only known way to slow the progression of the disease is to abstain from all tobacco products.