- Author: Naiem Nassiri, MD; Chief Editor: Vincent Lopez Rowe, MD more...
Thromboangiitis obliterans (TAO), an inflammatory vasculopathy also known as Buerger disease, is characterized by an inflammatory endarteritis that causes a prothrombotic state and subsequent vaso-occlusive phenomena. The inflammatory process is initiated within the tunica intima. It characteristically affects small and medium-sized arteries as well as veins of the upper and lower extremities. The condition is strongly associated with heavy tobacco use, and disease progression is closely linked to continued use. (See Pathophysiology and Etiology.)
Patients often present with moderate-to-severe claudication that can quickly progress to critical limb ischemia featuring rest pain or tissue loss. Features of acute limb ischemia (eg, pain, paresthesia, palor, mottling, poikilothermia, paresis, and pulselessness) are common signs and symptoms encountered in the emergency setting.[1, 2, 3, 4] (See Presentation.)
Pharmacologic therapy is generally ineffective; abstinence from tobacco is the only measure known to prevent disease progression. (See Treatment.) Given the arteritis of the small and medium-sized vessels, surgical or endovascular revascularization may not be possible, because of the absence of a distal target for revascularization. As the disease evolves, amputation may be the only viable option.
Pathophysiology and Etiology
As noted, the development of TAO is strongly associated with heavy use of tobacco, and the progression of the disease is closely linked to continued use.
A few observations suggest the existence of an immunologic phenomenon leading to vasodysfunction and inflammatory thrombi. Patients with TAO exhibit hypersensitivity to intradermally injected tobacco extracts, increased cellular sensitivity to collagen types I and III, elevated serum anti–endothelial cell antibody titers, and impaired peripheral endothelium-dependent vasorelaxation. They also show a higher prevalence of human leukocyte antigen (HLA)–A9, HLA-A54, and HLA-B5, suggesting a genetic component to the disease.
United States statistics
The prevalence of TAO has decreased over the past 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. Since then, the prevalence has fallen to an estimated 12.6-20 cases per 100,000 population.
Age-, sex-, and race-related demographics
Most patients with TAO are aged 20-45 years; the disease does not occur in pediatric or elderly patients. TAO is more common in males (male-to-female ratio, 3:1); however, the incidence in women is believed to be increasing, probably as a consequence of the growing frequency of smoking among women. The disease is relatively less common in people of northern European descent; natives of India, Korea, and Japan, along with Israeli Jews of Ashkenazi descent, have the highest incidence of TAO.
Death from TAO is rare. Between 1999 and 2007, according to data from the US Centers for Disease Control and prevention (CDC), TAO (code I73.1 in the International Classification of Diseases, Tenth Revision [ICD-10]) was the underlying cause of 117 deaths in the United States.
A striking dichotomy is observed in the prognosis of patients with TAO, which is dependent on whether absolute avoidance of tobacco is achieved. Among patients who stop using tobacco, 94% avoid amputation; among patients who stop using tobacco before progression to critical limb ischemia, the amputation rate is near 0%. In stark contrast, among patients who continue using tobacco, there is an 8-year amputation rate of 43%.
Patients with TAO must be repeatedly advised to cease all use of or exposure to tobacco products (including chewing tobacco, nicotine patches and gums, and second-hand smoke) and reassured that if they are able to discontinue tobacco use, the disease will remit and amputation will be avoided.
Physicians should counsel patients that the level of tobacco avoidance required to achieve resolution of their disease often necessitates that they rigorously limit their exposure even to secondhand smoke. This can be extremely difficult for patients who live with another smoker, and it is therefore not unreasonable to consider referring such patients (and their loved ones) to multidisciplinary smoking cessation programs.
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|Positive Criterion||Positive Points|
|Age at onset||< 30 y (+2)
30-40 y (+1)
|Foot intermittent claudication||Present (+2)
By history only (+1)
|Upper extremity||Symptomatic (+2)
|Migrating superficial thrombophlebitis||Present (+2)
By history only (+1)
|Raynaud phenomenon||Present (+2)
By history only (+1)
|Angiography; biopsy||If typical, both (+2)
|Negative Criterion||Negative Points|
|Age at onset||45-50 y (−1)
>50 y (−2)
|Sex; smoking||Female (−1)
|Location||Single limb (−1)
No lower extremity involved (−2)
|Absent pulses||Brachial (−1)
|Arteriosclerosis, diabetes, hypertension, hyperlipidemia||Discovered 5.1-10 y after diagnosis (−1)
Discovered 2.1-5 y later (−2)
|No. of Points||Probability of Diagnosis of Thromboangiitis Obliterans|
|2-3||Diagnosis suspected (low probability)|
|4-5||Diagnosis probable (medium probability)|
|≥6||Diagnosis definite (high probability)|