Buerger’s disease occurs throughout the world, though it is more prevalent in Asian and Mediterranean populations. Black populations are only rarely affected. Buerger’s disease was once thought to only occur in men typically below 45 years of age, with historical data demonstrating only 1 to 2% of all Buerger patients to be women. More recent series have revealed a notable increase in the number of women affected, with 8 to 23% of Buerger patients being women. The exact reason for this change is uncertain, although there has been a significant increase in the use of tobacco by women over the past several decades.

Furthermore, a substantial decline in the prevalence of Buerger’s disease has also been noted over the past few decades, in both the United States
and Japan. A Mayo clinic series noted that despite an increase in patient enrollment overall, the rate of Buerger’s disease steadily decreased from 104 per 100,000 in 1947 to only 13 cases per 100,000 in 1986. In Japan, Matsushita et al. have noted a similar trend. In a review of their extensive national registry of Buerger patients from 1989 to 1996, they noted that 46 new cases were diagnosed between 1985 and 1989, while during the period between 1990 and 1996, only 12 new cases were diagnosed. This decline likely represents refinements in the diagnosis of Buerger’s disease with the widespread use of angiography, though other causative factors may exist, including the general trend toward decreased use of tobacco.

Although the cause of Buerger’s disease remains unknown, there is a clear and well-established association between Buerger’s disease and tobacco exposure. In fact, many experts believe that nonexposure to tobacco (smoking or chewing) effectively excludes the diagnosis of Buerger’s disease. Even passive exposure to tobacco may trigger disease activity and progression. In keeping with this history, a number of patients with Buerger’s disease have poor oral hygiene and periodontal infections typically with anaerobic bacteria.

The exact mechanism by which tobacco causes Buerger’s disease is not known. There is some suggestion that tobacco use may damage the vascular endothelium with subsequent development of autoantibodies to the endothelium. In addition, deposition of autoantibodies, complement C3, and immune complexes have been demonstrated within vessel walls of patients with Buerger’s disease, as have T-lymphocytes exhibiting cellular sensitivity to type II and type III collagens, further suggesting an immune-mediated pathogenesis. Buerger’s disease has also been linked to exposures to other substances including arsenic, cocaine, and cannabis, though the exact nature of these associations remains uncertain. The prothrombin gene mutation 20210 and the presence of anticardiolipin antibodies are associated with an increased risk of the disease. Thromboangiitis obliterans patients with high anticardiolipin antibody titers tend to be younger and are thought to be at an increased risk for major amputation.

Buerger’s disease patients typically tend to be male, between 20 and 45 years of age at the time of onset. Though previously thought to be uncommon, women are increasingly being diagnosed. Exposure to tobacco products is universal, with most being heavy smokers but chewing exposure to tobacco as well as marijuana use has been reported. Patients typically present with evidence of arterial insufficiency affecting the extremities. Though the clinical presentation can vary depending on the severity of disease, a review of three large clinical series (Table 9.1) concludes that claudication, rest pain, and ulceration are often the initial manifestations of this disease. Disease
begins in the small vessels, resulting in symptoms of distal ischemia. In fact, claudication is most commonly seen in the foot and instep as compared to the classic calf claudication seen in patients with peripheral arterial disease. Ulceration is also a common initial presenting symptom, with ischemia involving the distal tip of the effected digit. Proximal disease progression or the development of superinfection are typical scenarios.

In contrast to atherosclerotic arterial disease, the upper extremities are often involved in Buerger’s disease. Involvement of multiple limbs is also a frequent finding. Shionoya evaluated 255 patients with Buerger’s disease seen over 12 years, and found that more than 84% of patients had involvement of three or four extremities and no cases had single extremity involvement. Venous involvement is highly variable and may precede arterial manifestations in a minority of patients, with migratory phlebitis being the most common presentation.

Although Buerger’s disease typically presents with distal manifestations, proximal progression is the rule in the face of continued exposure to tobacco. Abstinence from all tobacco products ensures a benign course. Amputation may often be required, occurring in up to 25% of affected patients. Although Buerger’s disease is primarily localized to the extremities, in isolated cases visceral, coronary, and cerebral thromboangiitis

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