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Buerger Disease (Thromboangiitis Obliterans)
Updated: May 1, 2009
Introduction
Background
Buerger disease, a nonatherosclerotic vascular disease also known as thromboangiitis obliterans (TAO), is characterized by the absence or minimal presence of atheromas, segmental vascular inflammation, vasoocclusive phenomenon, and involvement of small- and medium-sized arteries and veins of the upper and lower extremities. The condition is strongly associated with heavy tobacco use, and progression of the disease is closely linked to continued use. The typical presentations are rest pain, unremitting ischemic ulcerations, and gangrene of the digits of hands and feet, and as the disease evolves, the patients may require several surgical amputations.1,2
The first reported case of thromboangiitis obliterans was described in Germany by von Winiwarter in an 1879 article titled "A strange form of endarteritis and endophlebitis with gangrene of the feet."3 A little more than a quarter of a century later, in Brookline, NY, Leo Buerger published a detailed description of the disease in which he referred to the clinical presentation of thromboangiitis obliterans as "presenile spontaneous gangrene."4 The paper discussed the pathological findings in 11 limbs amputated from Jewish patients with the disease.
The feet of a patient with Buerger disease. Note the ischemic ulcers on the distal portion of the left great, second, and fifth toes. Though the patient's right foot is normal in gross appearance, angiography demonstrated compromised arterial flow to both feet.
Pathophysiology
While the etiology of Buerger disease is unknown, exposure to tobacco is essential for both initiation and progression of the disease. The notion that the condition is linked to tobacco exposure is supported by the fact that the disease is more common in countries with heavy use of tobacco and is perhaps most common among natives of Bangladesh who smoke a specific type of cigarettes, homemade from raw tobacco, called "bidi." While the overwhelming majority of patients with Buerger disease smoke, a few cases have been reported in nonsmokers that have been attributed to the use of chewing tobacco.
The disease mechanism underlying Buerger disease remains unclear, but a few observations have led investigators to implicate an immunologic phenomenon that leads to vasodysfunction and inflammatory thrombi. Patients with the disease show hypersensitivity to intradermally injected tobacco extracts, have increased cellular sensitivity to types I and III collagen, have elevated serum anti–endothelial cell antibody titers, and have impaired peripheral vasculature endothelium-dependent vasorelaxation. Increased prevalence of HLA-A9, HLA-A54, and HLA-B5 is observed in these patients, which suggests a genetic component to the disease.
Frequency
United States
The prevalence of Buerger disease has decreased over the past half decade, partly because the prevalence of smoking has decreased, but also because the diagnostic criteria have become more stringent. In 1947, the prevalence of the disease in the United States was 104 cases per 100,000 population. More recently, prevalence has been estimated at 12.6-20 cases per 100,000 population.
Mortality/Morbidity
Death from Buerger disease is rare, but in patients with the disease who continue to smoke, 43% require 1 or more amputations in 7.6 years. Most recently, in a December 2004 CDC publication, the 2002 deaths report in the United States divided by cause of death, month, race, and sex (based on the International Classification of Diseases, Tenth Revision, 1992), reported a total of 9 deaths related to TAO, depicting male to female gender ratio of 2:1 and white to black ethnicity ratio of 8:1.
Race
Buerger disease is relatively less common in people of northern European descent. Natives of India, Korea, and Japan, and Israeli Jews of Ashkenazi descent, have the highest incidence of the disease.5
Sex
Though Buerger disease is more common in males (male-to-female ratio, 3:1), incidence is believed to be increasing among women, and this trend is postulated to be due to the increased prevalence of smoking among women.
Age
Most patients with Buerger disease are aged 20-45 years.
Clinical
History
- Because a firm diagnosis of Buerger disease is difficult to establish, a number of different diagnostic criteria have been proposed.6 Olin asserts that the following criteria must be met for the diagnosis to be made with reasonable certainty:7
- Age younger than 45 years
- Current (or recent) history of tobacco use
- Presence of distal extremity ischemia (indicated by claudication, pain at rest, ischemic ulcers, or gangrene) documented by noninvasive vascular testing
- Exclusion of autoimmune diseases, hypercoagulable states, and diabetes mellitus by laboratory tests
- Exclusion of a proximal source of emboli by echocardiography and arteriography
- Consistent arteriographic findings in the clinically involved and noninvolved limbs
- Most patients (70-80%) with Buerger disease present with distal ischemic rest pain and/or ischemic ulcerations on the toes, feet, or fingers, as depicted in the images below.8
The feet of a patient with Buerger disease. Note the ischemic ulcers on the distal portion of the left great, second, and fifth toes. Though the patient's right foot is normal in gross appearance, angiography demonstrated compromised arterial flow to both feet.
- Progression of the disease may lead to involvement of more proximal arteries, but involvement of large arteries is unusual.
- Patients may also present with claudication of the feet, legs, hands, or arms and often describe the Raynaud phenomenon of sensitivity of the hands and fingers to cold.
- Foot or arch claudication may be erroneously attributed to an orthopedic problem.
- Patients who seek medical attention late in the course of their disease may present with foot infections and, occasionally, with florid sepsis.
- Although classic Buerger disease affects the vessels of the extremities, a few cases of aortic, cerebral, coronary, iliac, mesenteric, pulmonary, and renal thromboangiitis obliterans have been reported.
Physical
- Patients with Buerger disease develop painful ulcerations and/or frank gangrene of the digits.
- The hands and feet of patients with the disease are usually cool and mildly edematous.
- Superficial thrombophlebitis (often migratory) occurs in almost half of patients with Buerger disease.
- Paresthesias (numbness, tingling, burning, hypoesthesia) of the feet and hands and impaired distal pulses in the presence of normal proximal pulses are usually found in patients with the disease.
- More than 80% percent of patients present with involvement of 3-4 limbs.
- More recently a point-scoring system has been proposed by Papa to support or contest the diagnosis of TAO using the following criteria.
- Distal extremity (feet, toes, hands, fingers) involvement
- Onset before age 45
- Tobacco use
- Exclusion of atherosclerosis or proximal source of emboli
- Lack of hypercoagulable state
- Lack of definable arteritis (ie, progressive systemic sclerosis, giant cell arteritis)
- Classic arteriographic findings
- Involvement of digital arteries of finger or toes
- Segmental involvement (ie, "skip areas")
- Corkscrew collaterals
- No atherosclerotic changes
- Classic histopathologic findings
- Inflammatory cellular infiltrate within thrombus
- Intact internal elastic lamina
- Involvement of surrounding venous tissues
Table 1. Scoring system for the diagnosis of thromboangiitis obliterans (x)
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Table
| Positive points | |
| Age at onset | Less than 30 (+2)/30-40 years (+1) |
| Foot intermittent claudication | Present (+2)/ by history (+1) |
| Upper extremity | Symptomatic (+2)/ asymptomatic (+1) |
| Migrating superficial vein thrombosis | Present (+2)/ by history only (+1) |
| Raynaud | Present (+2)/ by history only (+1) |
| Angiography; biopsy | If typical both (+2)/ either(+1) |
| Negative points | |
| Age at onset | 45-50 (-1)/more than 50 years (-2) |
| Sex, smoking | Female (-1)/ nonsmoker (-2) |
| Location | Single limb (-1)/no LE involved (-2) |
| Absent pulses | Brachial (-1)/femoral (-2) |
| Arteriosclerosis, diabetes, hypertension, hyperlipidemia | Discovered after diagnosis 5.1-10 years (-1)/2.1- 5 years later (-2) |
| Positive points | |
| Age at onset | Less than 30 (+2)/30-40 years (+1) |
| Foot intermittent claudication | Present (+2)/ by history (+1) |
| Upper extremity | Symptomatic (+2)/ asymptomatic (+1) |
| Migrating superficial vein thrombosis | Present (+2)/ by history only (+1) |
| Raynaud | Present (+2)/ by history only (+1) |
| Angiography; biopsy | If typical both (+2)/ either(+1) |
| Negative points | |
| Age at onset | 45-50 (-1)/more than 50 years (-2) |
| Sex, smoking | Female (-1)/ nonsmoker (-2) |
| Location | Single limb (-1)/no LE involved (-2) |
| Absent pulses | Brachial (-1)/femoral (-2) |
| Arteriosclerosis, diabetes, hypertension, hyperlipidemia | Discovered after diagnosis 5.1-10 years (-1)/2.1- 5 years later (-2) |
Table 2. Sum of points defines the probability of the diagnosis of thromboangiitis obliterans
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Table
| Number of points | Probability of diagnosis |
| 0-1 | Diagnostic excluded |
| 2-3 | Suspected, low probability |
| 4-5 | Probable, medium probability |
| 6 or more | Definite, high probability |
| Number of points | Probability of diagnosis |
| 0-1 | Diagnostic excluded |
| 2-3 | Suspected, low probability |
| 4-5 | Probable, medium probability |
| 6 or more | Definite, high probability |
Causes
Propagating agents include cigarettes, as depicted in the image below, chewing tobacco, nicotine patches, and secondhand tobacco smoke (the latter two have been implicated as propagating agents of the disease only in former smokers).
The tobacco smoke–stained fingers of this patient suggested the man's diagnosis (Buerger disease). The patient presented with small, painful ulcers on the tips of his thumb and ring finger.
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| Treatment & Medication: Buerger Disease (Thromboangiitis Obliterans) |
| Follow-up: Buerger Disease (Thromboangiitis Obliterans) |
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| References |
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References
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Further Reading
Clinical guidelines
Counseling to prevent tobacco use and tobacco-caused disease: recommendation statement.
United States Preventive Services Task Force - Independent Expert Panel. 1996 (revised 2003 Nov). 13 pages. NGC:003268
Chronic wounds of the lower extremity.
American Society of Plastic Surgeons - Medical Specialty Society. 2007 May. 21 pages. NGC:005966
VA/DoD Clinical practice guideline for rehabilitation of lower limb amputation.
Department of Defense - Federal Government Agency [U.S.]
Department of Veterans Affairs - Federal Government Agency [U.S.]
Veterans Health Administration - Federal Government Agency [U.S.]. 2007 Aug. 163 pages. NGC:006060
Clinical trial
TACT-NAGOYA: Therapeutic Angiogenesis Using Cell Transplantation
Related eMedicine topics
Digital Amputations
Atherosclerosis
Vascular Occlusive Syndromes of the Upper Extremity
Vascular Ulcers
Keywords
Buerger disease, Buerger's disease, thromboangiitis obliterans, TAO, endarteritis, endophlebitis, vasoocclusive disease, presenile spontaneous gangrene, corkscrew collaterals, bidi, cigarette smoking, tobacco use, secondhand smoke, smoking cessation, distal ischemic rest pain, ischemic ulcerations, amputation






Overview: Buerger Disease (Thromboangiitis Obliterans)