Thromboangiitis obliterans, also known as Buerger's disease, is a nonatherosclerotic inflammatory disease of the small-sized and medium-sized arteries and veins of the arms and legs. Although inflammatory by nature, it spares the blood vessel walls. Unlike other types of vasculitis, the acute-phase reactants (such as the erythrocyte sedimentation rate and C-reactive protein) are normal in patients with Buerger's disease. A pathologic finding of acute disease is an occlusive, highly cellular, inflammatory thrombus. The disease is found worldwide, but the highest incidence of thromboangiitis obliterans occurs in the Middle East and Far East. Prevalence ranges from about 12 percent in the United States to 63 percent in India to 80 percent among Jews of Ashkenazi ancestry living in Israel. Part of this difference in disease incidence may be due to variability in diagnostic criteria. Olin reviewed the current concepts of this disease.
Although the cause of Buerger's disease is unknown, the strongest association is with tobacco use. Occurrences of the disease have been reported, but uncommonly, in persons who use smokeless tobacco or snuff. A current or previous history of smoking is usually a required diagnostic criterion.
The onset of Buerger's disease occurs between 40 and 45 years of age, and men are most commonly affected. It begins with ischemia of the distal small vessels of the arms, legs, hands and feet (see accompanying table). Patients often present with claudications in affected limbs and progress to ischemic pain at rest and ulcerations of the toes or fingers. Claudications in the foot or arch may be misdiagnosed as an orthopedic problem. More proximal arteries may bcome involved as the disease progresses.
There are no specific serologic markers to diagnose Buerger's disease. Recommended tests to rule out other causes of vasculitis include a complete blood cell count, liver function tests, serum creatinine determination, fasting blood sugar level, sedimentation rate, antinuclear antibody, rheumatoid factor, serologic markers for CREST syndrome and scleroderma, and screening for hypercoagulability. If a proximal source of embolization is suspected, transthoracic or transesophageal echocardiography and arteriography should be performed. Angiographic findings include severe distal, segmental occlusive lesions, but the more proximal arteries (below the popliteal and distal to brachial branches) are normal.
The only truly effective treatment for thromboangiitis obliterans is smoking cessation or discontinuation of tobacco-containing products. In one study, 94 percent of affected persons who quit smoking avoided amputations compared with 57 percent who continued to smoke. Therapy with intravenous iloprost (a prostaglandin analog) has been studied. This agent was found to be superior to aspirin in producing clinical improvement and decreasing the need for amputation. It is not currently available in the United States. Experience with intra-arterial thrombolytic therapy is limited to a few trials that have shown moderate success, but more studies are needed. Surgical revascularization is not feasible because of the diffuse segmental involvement and the distal nature of the vascular involvement.
|Age younger than 45 years|
|Current or recent history of tobacco use|
|Presence of distal extremity ischemia (claudications, pain at rest, ischemic ulcers)|
|Exclusion of autoimmune diseases, hypercoagulable states and diabetes mellitus|
|Exclusion of a proximal source of embolization by echocardiography and arteriography|
|Consistent arteriographic findings in the clinically involved and noninvolved limbs|