Buerger Disease

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Buerger Disease (Thromboangiitis Obliterans) 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 mediumsized 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. Th
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  Buerger Disease (Thromboangiitis Obliterans) Buerger disease, a nonatherosclerotic vascular disease also known as thromboangiitisobliterans (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 associatedwith 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 surgicalamputations. [1, 2]  The first reported case of thromboangiitis obliterans was described in Germany by vonWiniwarter in an 1879 article titled A strange form of endarteritis and endophlebitis withgangrene 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 clinicalpresentation of thromboangiitis obliterans as presenile spontaneous gangrene. [4] The paperdiscussed the pathological findings in 11 limbs amputated from Jewish patients with thedisease. The feet of a patient with Buerger disease. Note the ischemic ulcers on thedistal portion of the left great, second, and fifth toes. Though the patient's right foot is normal in grossappearance, 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 useof 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 observationshave led investigators to implicate an immunologic phenomenon that leads to vasodysfunctionand inflammatory thrombi. Patients with the disease show hypersensitivity to intradermally   injected tobacco extracts, have increased cellular sensitivity to types I and III collagen, haveelevated 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. Epidemiology  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 havebecome 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 CDCpublication, 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 toblack 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 ofthe disease. [5]     Sex Though Buerger disease is more common in males (male-to-female ratio, 3:1), incidence isbelieved to be increasing among women, and this trend is postulated to be due to the increasedprevalence of smoking among women. Age Most patients with Buerger disease are aged 20-45 years. History    Because a firm diagnosis of Buerger disease is difficult to establish, a number of differentdiagnostic 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 bylaboratory 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 thedistal portion of the left great, second, and fifth toes. Though the patient's right foot is normal in grossappearance, angiography demonstrated compromised arterial flow to both feet.Superficial thrombophlebitis of the great toe in a patient with Buerger disease.      Progression of the disease may lead to involvement of more proximal arteries, but involvementof 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 footinfections 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 beenreported.   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 thedisease.    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 thediagnosis 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 tissuesTable 1. Scoring system for the diagnosis of thromboangiitis obliterans (x)(Open Table in a newwindow)    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,hyperlipidemiaDiscovered after diagnosis 5.1-10 years (-1)/2.1- 5years later (-2) Table 2. Sum of points defines the probability of the diagnosis of thromboangiitisobliterans(Open Table in a new window)    Number of points   Probability of diagnosis  0-1 Diagnostic excluded
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