Buerger's disease


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Related to Buerger's disease: Addison's disease, Berger's disease

Buerger's Disease

 

Definition

Buerger's disease is an inflammation of the arteries, veins, and nerves in the legs, principally, leading to restricted blood flow. Left untreated, Buerger's disease can lead to gangrene of the affected areas. Buerger's disease is also known as thromboangitis obliterans.

Causes and symptoms

The exact cause of Buerger's disease is not known. It is seen most often in young to middle-aged men (ages 20-40) who are heavy smokers of cigarettes. Cases of this disease in non-smokers are very rare, hence, cigarette smoking is considered a causative factor. Approximately 40% of the patients have a history of inflammation of a vein (phlebitis), which may play a role in the development of Buerger's disease. The disease is mainly seen in the legs of affected persons, but may also appear in their arms. Early symptoms include decrease in the blood supply (arterial ischemia) and superficial (near the skin surface) phlebitis. The main symptom is pain in the affected areas. Onset of the disease is gradual and first occurs in the feet or hands. Inflammation occurs in small and medium-sized arteries and veins near the surface of the limb. In advanced cases, blood vessels in other parts of the body may be affected. There is a progressive decrease in the blood flow to the affected areas. The pulse in arteries of the feet is weak or undetectable. The lack of blood flow can lead to gangrene, which is decay of tissue due to restricted blood supply. A cold sensitivity in the hands, similar to that seen in Raynaud's disease, can develop. In this case, the hands turn color—white, blue, and then red—when exposed to the cold.

Diagnosis

Diagnosis is usually made from the clinical symptoms. Patients frequently complain of numbness, tingling, or burning sensations in the affected area before evidence of vascular inflammation becomes apparent.

Treatment

There is no effective medication or surgery for this disease. Patients must stop smoking to halt further development of the symptoms. Vasodilators, drugs that increase the diameter of the blood vessels, can be administered, but may not be effective. Exposure of affected areas to heat or cold should be avoided. Trauma to the feet and other affected areas should be avoided and infections must be treated promptly.

Prognosis

The disease is progressive in patients who do not stop smoking. Areas with gangrene must be removed surgically.

Prevention

Smoking is the only known causative agent for this disease and should be avoided.

Resources

Books

Berkow, R., editor. The Merck Manual. 17th ed. Rahway, NJ: Merck and Co., 1997.

Key terms

Gangrene — A decay of the tissue in a part of the body that experiences restricted blood flow.
Inflammation — A local reaction to irritation, injury, or infection characterized by pain, swelling, redness, and occasional loss of function.
Ischemia — A decrease in the blood supply to an area of the body caused by obstruction or constriction of blood vessels.
Phlebitis — Inflammation of a vein.

thromboangiitis

 [throm″bo-an″je-i´tis]
inflammation of a blood vessel (vasculitis) with thrombosis.
thromboangiitis obli´terans a disease affecting the medium-sized blood vessels, particularly the arteries of the lower limbs, which can cause severe pain and in serious cases lead to gangrene. The name refers to the clotting, pain, and inflammation occurring in this disease and to the fact that it can obliterate, or destroy, blood vessels. The cause has been thought to be excessive use of tobacco over a long period of time, and the number of cases has diminished strikingly in recent years. The intense pain is caused by thrombosis (formation of blood clots) in the lining of the arteries; when the clots grow larger, the blood flow slows and may stop entirely. Since every part of the body depends on the continuous flow of blood, affected areas such as fingers and toes, for example, soon begin to atrophy or develop ulcers. If the causes of the disease are not completely arrested, amputation may be necessary.



To treat the disease, the patient must stop smoking at once and entirely. This generally results in the partial healing of the affected membrane with a renewed flow of blood. However, more blood may have to be brought to damaged tissue by surgical methods of channeling detours or making canals in the clot itself. Sympathectomy may be tried to prevent any vasospastic component. Minor amputations of gangrenous tissue are often needed.

Special exercises called buerger-allen exercises are sometimes used to empty the engorged blood vessels and stimulate collateral circulation. These exercises can be done at home by patients and are usually prescribed to be done several times during the day. Patients are also instructed to avoid wearing any tight clothing, such as tight girdles, rolled garters, constricting belts, and other items that may impair circulation. They should also avoid sitting or standing in one position for long periods of time. Care should be used in the selection of shoes and stockings so that they fit properly and do not cause pressure against the blood vessels. Patients should be told to avoid walking barefoot or otherwise subjecting themselves to the hazards of trauma to the feet and legs. Should such an accident occur, no matter how minor it may seem, the patient must notify a health care provider so that treatment may be begun and infection and ulceration can be prevented.

Buerger's disease

An arterial disease of male smokers featuring obstruction of arteries, especially those supplying the legs, followed by gangrene necessitating amputation. It is also called thromboangiitis obliterans. Sufferers often continue to smoke. (Leo Buerger, 1879–1943, American physician).
References in periodicals archive ?
This case reinforces the age-old aphorism of "no tobacco, no Buerger's disease." (4)
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All the CLI patients screened had an underlying aetiology due to atherosclerotic arterial occlusive disease (PAD) or Thromboangiitis Obliterans (TAO)/ Buerger's disease. Out of the 22 patients screened, 17 patients met the eligibility criteria and were enrolled in the study.
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Even if the patient is young he was diagnosed with PAD instead of Buerger's disease, which was more likely for this case because of the smoking history, the age and the lesion.
The differential diagnosis should include other, more common, causes of ischemic hands: Buerger's disease, primary and secondary Raynaud's phenomenon, systemic vasculitis, cardiac embolism, pulmonary arterio-arterial, arterio-arterial embolism, congenital or acquired hypercoagulability (thrombophilia), and thoracic outlet syndrome.
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The company also received orphan drug status from the FDA for its PLX cells for the treatment of Buerger's disease in August of 201 1.
Pluristem is targeting several therapeutics indications for which clinical trials and preclinical studies are in progress, which include: intermittent claudication, muscle regeneration critical limb ischemia, Buerger's disease, and acute radiation syndrome.