12-08: Thromboangiitis Obliterans (Buerger Disease)

GENERAL CONSIDERATIONS

Buerger disease is a segmental, inflammatory, and thrombotic process of the distal-most arteries and occasionally veins of the extremities. Pathologic examination reveals arteritis in the affected vessels. The cause is not known but it is rarely seen in nonsmokers. Arteries most commonly affected are the plantar and digital vessels of the foot and lower leg. In advanced stages, the fingers and hands may become involved. The incidence of Buerger disease has decreased dramatically.

CLINICAL FINDINGS

A. Symptoms and Signs

Buerger disease may be initially difficult to differentiate from routine peripheral vascular disease, but in most cases, the lesions are on the toes and the patient is younger than 40 years of age. The observation of superficial thrombophlebitis may aid the diagnosis. Because the distal vessels are usually affected, intermittent claudication is not common with Buerger disease, but rest pain, particularly pain in the distal most extremity (ie, toes), is frequent. This pain often progresses to tissue loss and amputation, unless the patient stops smoking. The progression of the disease seems to be intermittent with acute and dramatic episodes followed by some periods of remission.

B. Imaging

MRA or invasive angiography can demonstrate the obliteration of the distal arterial tree typical of Buerger disease.

DIFFERENTIAL DIAGNOSIS

In atherosclerotic peripheral vascular disease, the onset of tissue ischemia tends to be less dramatic than in Buerger disease, and symptoms of proximal arterial involvement, such as claudication, predominate.

Symptoms of Raynaud disease may be difficult to differentiate from Buerger disease and are often coexistent in 40% of patients. Repetitive atheroemboli may also mimic Buerger disease and may be difficult to differentiate. It may be necessary to image the proximal arterial tree to rule out sources of arterial microemboli.

TREATMENT

Smoking cessation is the mainstay of therapy and will halt the disease in most cases. As the distal arterial tree is occluded, revascularization is often not possible. Sympathectomy is rarely effective.

PROGNOSIS

If smoking cessation can be achieved, the outlook for Buerger disease may be better than in patients with premature peripheral vascular disease. If smoking cessation is not achieved, then the prognosis is generally poor, with amputation of both lower and upper extremities a possible outcome.