12-09: Thromboangiitis Obliterans (Buerger Disease)

ESSENTIALS OF DIAGNOSIS

GENERAL CONSIDERATIONS

Thromboangiitis obliterans (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 patients who do not smoke cigarettes. 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 thromboangiitis obliterans has decreased dramatically.

CLINICAL FINDINGS

A. Symptoms and Signs

Thromboangiitis obliterans may be initially difficult to differentiate from atherosclerotic peripheral vascular disease, but in most cases, the lesions are on the toes and the patient is younger than 40 years. The observation of superficial thrombophlebitis may aid the diagnosis. Because the distal vessels are usually affected, intermittent claudication is not common, 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 thromboangiitis obliterans.

DIFFERENTIAL DIAGNOSIS

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

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

TREATMENT

Cessation of cigarette smoking 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. Intra-arterial infusion of prostacyclin analogs has been reported to improve ulcer healing in select cases. Sympathectomy is rarely effective.

PROGNOSIS

If smoking cessation can be achieved, the outlook for thromboangiitis obliterans 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.