Buerger's Disease

Buerger's Disease

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Buerger's Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Inflammatory Occlusive Peripheral Vascular Disease
  • Occlusive Peripheral Vascular Disease
  • Thromboangiitis Obliterans
  • TAO

Disorder Subdivisions

  • None

General Discussion

Buerger's disease, also known as thromboangiitis obliterans, is a rare disorder that, in most cases, affects young or middle-aged male cigarette smokers. It is characterized by narrowing or blockage (occlusion) of the veins and arteries of the extremities, resulting in reduced blood flow to these areas (peripheral vascular disease). The legs are affected more often than the arms. In most cases, the first symptom is extreme pain of the lower arms and legs while at rest. Affected individuals may also experience cramping in the legs when they walk that, in rare cases, may cause limping (claudication). In addition, affected individuals may have sores (ulcers) on the extremities, numbness and tingling and a lack of normal blood flow to the fingers and/or toes when exposed to cold temperatures (Raynaud's phenomenon), and/or inflammation and clotting of certain veins (thrombophlebitis). In severe cases, individuals with Buerger's disease may exhibit tissue death (gangrene) of affected limbs. The exact cause of Buerger's disease is not known; however, most affected individuals are heavy tobacco users.

Symptoms

Buerger's disease is characterized by narrowing or blockage (occlusion) of the intermediate and/or small sized arteries and veins of the extremities, resulting in reduced blood flow to these areas (peripheral vascular disease). Buerger's disease tends to occur in sudden (acute) episodes that may last from one to four weeks. The disorder runs a recurrent course.



In most cases, the first sign of Buerger's disease is extreme pain in the lower arms and/or legs while at rest. Affected individuals may also experience cramping in the legs when they walk that, in rare cases, may cause affected individuals to limp (claudication). Other physical features associated with Buerger's disease may include sores (ulcers) on the arms and legs, pale discoloration of the hands, numbness and tingling and a lack of normal blood flow to the fingers and/or toes when exposed to cold temperatures (Raynaud's phenomenon), and inflammation and clotting of certain veins (thrombophlebitis). Dry dark ulcerations that often form on the tips of the fingers or toes may be extremely painful. Pain associated with these ulcers may worsen with elevation. In severe cases, individuals with Buerger's disease may exhibit tissue death (gangrene) of the affected areas.



In some cases, arteries and veins of the intestines may also be affected. This may result in extreme heaviness or pain (angina) in the abdomen and weight loss. In extremely rare cases (i.e., fewer than 2 percent), affected individuals have exhibited neurological abnormalities.

Causes

The exact cause of Buerger's disease is unknown. However, the use of tobacco is associated with the development of the disorder. In fact, most researchers believe that past or present use of tobacco products by an individual is a requirement for the diagnosis of Buerger's disease. The exact relationship tobacco products have with Buerger's disease is not completely understood.



Some scientists believe that Buerger's disease may be an autoimmune disorder. In some cases, trauma to the hands and feet may contribute to the disorder. Autoimmune disorders are caused when the body's natural defenses against "foreign" or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons.



Genetic factors may play a role in the development or severity of Buerger's disease because the prevalence varies significantly between ethic groups. More research is necessary to determine the exact role, if any, that genetics plays in the development of the disorder.



The symptoms of Buerger's disease develop because of impaired blood flow (ischemia) to certain areas of the body most often the arms and legs.

Affected Populations

Buerger's disease is a very rare disorder that, in most cases, affects young or middle-aged male cigarette smokers with onset of symptoms before 40-45 years of age. In recent years, more affected women have been reported in the medical literature. Some scientists have speculated that this is due to an increase in the amount of women who smoke. At one time the ratio of affected men to women was 100:1, recent articles in the medical literature have speculated that ratio may be 10:1 or closer. In extremely rare cases, individuals who do not smoke have developed Buerger's disease.



Buerger's disease is extremely rare in the United States and Europe, but more common in other parts of the world, especially in parts of Asia and the Far and Middle East. The incidence in the United States has been estimated at 12.6-20 per 100,000 people in the general population. Buerger's disease occurs with greater frequency in countries that have heavy tobacco use.



Although most cases are associated with cigarette smoking, the disorder has also developed in individuals who did not smoke but used smokeless tobacco (e.g., chewing tobacco).

Standard Therapies

Diagnosis

A diagnosis of Buerger's disease may be made based upon the identification of characteristic physical features and symptoms. Many physicians require a history of recent or current tobacco use in a diagnosis of Buerger's disease. A test such as an angiography or noninvasive techniques may be used to confirm a diagnosis. During an angiography, injection of a specialized dye is used to make the blood vessels visible on x-rays.



Treatment

The treatment of Buerger's disease is symptomatic and supportive. Symptoms usually improve if affected individuals stop smoking. In some cases, when individuals stop smoking complete remission of the disorder may occur.



If an affected individual does not stop smoking, treatment options for Buerger's disease should avoid premature or unnecessary surgery. Conservative treatment may include drugs that prevent the blood from clotting (anticoagulants), drugs that increase the diameter of blood vessels (vasodilators), drugs that prevent inflammation (antiinflammatories), antibiotics, and/or drugs that relieve pain (analgesics).



In some cases surgery may become necessary. Affected individuals have been treated with an operation in which nerve terminals (ganglia) are destroyed to interrupt the nerve pathway and improve blood supply to the affected limb (sympathectomay). In some cases, surgeons may try bypass procedures to get around the blocked or narrow (occluded) veins and/or arteries. In severe cases, surgeons may be forced to remove (amputate) a finger or toe or part of an arm or leg.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com



Revascularization is a type of experimental surgery being investigated as a treatment for Buerger's disease. This type of surgery on veins and arteries may enhance blood circulation if combined with drug therapy (urokinase, PGE1 and heparin), but more research is necessary to determine its safety and long-term effectiveness for individuals with Burger's disease.



Trials have been conducted to test the usefulness of a prostacyclin analogue known as iloprost and a surgical procedure known as omental transfer in the treatment of Buerger's disease. Iloprost alleviated pain and healed ulcers. Omental transfer helped to increase blood flow to the affected areas and alleviated pain and skin abnormalities. However, more studies are needed to determine the long-term safety and effectiveness or these options in the treatment of Buerger's disease.



Additional therapies are being studied for the treatment of Buerger's disease. Growth factors that stimulate angiogenesis to speed up healing and alleviate pain may be beneficial. Implantable nerve stimulators have been studied to treat pain associated with Buerger's disease.



In cases where other therapies have failed, a drug known as treprostinil sodium has been tried. Initial results demonstrate that this drug may be clinically useful for the treatment of affected individuals. More research is necessary to determine the long-term safety and effectiveness of this drug for the treatment of Buerger's disease.

References

TEXTBOOKS

Cooper LT. Thromboangiitis Obliterans. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:36.



Magalini SI, et al., eds. Dictionary of Medical Syndromes. 4th ed.New York, NY: Lippincott-Raven Publishers; 1997:123.



JOURNAL ARTICLES

Fernandez B, Strootman D. The prostacyclin analog, treprostinil sodium, provides symptom relief in severe Buerger's disease -- a case report and review of the literature. Angiology. 2006;57:99-102.



Cooper LT, et al., A prospective, case-control study of tobacco dependence in thromboangiitis obliterans (Buerger's disease). Angiology. 2006;57:73-8.



Olin JW, Shih A. Thromboangiitis obliterans. (Buerger's disease). Curr Opin Rheumatol. 2006;18:18-24.



Olin JW, Thromboangiitis Obliterans (Buerger's Disease). N Engl J Med. 2000;343:864-69.



Harten P, et al., Multiple organ manifestations in thromboangiitis obliterans (Buerger's disease). A case report. Anigology. 1996;47:419-25.



Singh I, et al., The role of omental transfer in Buerger's disease: New Delhi's experience. Aust N Z J Surg. 1996;66:372-76.



Puchmayer V, Clinical diagnosis, special characteristics and therapy of Buerger's disease. Bratisl Lek Listy. 1996;97:224-29.



Papa MZ, et al., A point scoring system for the diagnosis of Buerger's disease. Eur J Vasc Endovasc Surg. 1996;11:335-39.



Makita S, et al., Impaired endothelium-dependent vasorelaxation in peripheral vasculature of patients with thromboangiitis obliterans (Buerger's disease). Circulation. 1996;94:II211-25.



Njo KT, et al., Thromboangiitis obliterans (Buerger's disease) in 2 women. Ned Tijdschr Geneeskd. 1996;140:1770-72.



Nyuyen TB, et al., Buerger's disease disclosed by a perforation in the digestive system. Apropos of a case and review of the literature. Rev Med Interne. 1996:70-75.



Sauvaget F, et al., Colonic ischemia reveals thromboangiitis obliterans (Buerger's disease). Gastroenterology. 1996;110:900-03.



Ishibashi H, et al., Thoracoscopic sympathectomy for Buerger's disease: a report on the successful treatment of four patients. Surg Today. 1995;25:180-83.



Shindo S, et al., Collateral artery bypass in Buerger's disease: report of a novel procedure. Surg Today. 1995;25:92-95.



Treska V, et al., Surgical aspects of Buerger's disease. Bratisl Lek Listy. 1995;96:570-72.



Campello Morer I, et al., Thromboangiitis obliterans with cerebral involvement. Neurologia. 1995;10:384-86.



Sasajima T, et al., Plantar or dorsalis pedis artery bypass in Buerger's disease. Ann Vasc Surg. 1994;8:248-57.



Olin JW, Thromboangiitis obliterans. Curr Opin Rheumatol. 1994;6:44-49.



Schellong SM, et al., Intestinal type of thromboangiitis obliterans (Buerger's disease). J Intern Med. 1994;235:69-73.



Broide E, et al., Buerger's disease presenting as acute small bowel ischemia. Gastroenterology. 1993;104:1192-95.



Sayin A., et al., Surgical treatment of Buerger's disease: experience with 216 patients. Cardiovasc Surg. 1993;1:377-80.



Shionoya S, et al., Buerger's disease: diagnosis and management. Cardiovasc Surg. 1993;1:207-14.



Kempczinski RF, et al., Intestinal ischemia secondary to thromboangiitis obliterans. Ann Vasc Surg. 1993;7:354-58.



Fernandez-Miranda C, et al., Thromboangiitis obliterans (Buerger's disease). Study of 41 cases. Med Clin (Barc). 1993;101:321-26.



Yorukoglu Y, et al., Thromboangiitis obliterans (Buerger's disease) in women (a reevaluation). Angiology. 1993;44:527-32.



Grove WJ, et al., Buerger's disease and cigarette smoking in Bangladesh. Ann R Coll Surg Engl. 1992;74:115-18.



Papa M, et al., Autoimmune mechanisms in thromboangiitis obliterans (Buerger's disease): the role of tobacco antigen and the major histocompatibility complex. Surgery. 1992;111:527-31.



Lance BJ, et al., Distal ischemia with digital gangrene secondary to Buerger's disease. J Foot Surg. 1991;30:534-41.



Olin JW, et al., The changing clinical spectrum of thromboangiitis obliterans (Buerger's disease). Circulation. 1990;82:IV3-8.



Fiessinger JN, et al., Trial of iloprost versus aspirin treatment for critical limb ischaemia of thromboangiitis obliterans. The tao study. Lancet. 1990;335:555-57.



Ohta T, et al., Fate of the ischaemic limb in Buerger's disease. Br J Surg. 1988;75:259-62.



Lie JT, et al., Thromboangiitis obliterans with eosinophilia (Buerger's disease) of the temporal arteries. Hum Pathol. 1988;19:598-602.



Lie JT, et al., Thromboangiitis obliterans (Buerger's disease) in a saphenous vein arterail graft. Hum Pathol. 1987;18:402-04.



FROM THE INTERNET

Hanly EJ. Buerger's Disease (Thromboangiitis Obliterans). Last Updated: August 8, 2005. 9pp. Available at: http://www.emedicine.com/med/topic253.htm Accessed on: February 12, 2006.



Beers MH, Ed. The Merck Manual of Medical Information [book online]. Merck Research Laboratories. 2004-2005. Available at: http://www.merck.com/mrkshared/mmanual/section16/chapter212/212c.jsp Accessed on: February 12, 2006.

Resources

Vasculitis Foundation

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Vascular Disease Foundation

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Genetic and Rare Diseases (GARD) Information Center

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