Other Arteriopathies

Thromboangiitis Oblierans (Buerger's Disease)
Buerger's disease is an episodic and segmental inflammatory and thrombotic process of the peripheral arteries and veins. The cause is not known. It is seen most commonly in men under 40 years of age who smoke, and particularly in those of Eastern European or Asian background. It is characterized by occlusion of distal arteries, producing claudication, rest pain, and tissue necrosis. The inflammatory process is intermittent, with quiescent periods lasting weeks, months, or years. Different arterial segments may become occluded in successive episodes; recanalization can occur during periods of disease remission.

There are no pathognomonic signs, but several findings are characteristic of Buerger's disease. (1) The typical patient is a man under 40 years of age who smokes. Fewer than 20% of patients are women. (2) There is a history of migratory superficial segmental thrombophlebitis, eg, red, tender nodules in the branches of the saphenous vein. Biopsy of the affected vein may show inflammatory infiltrate in the vessel wall, microabscesses, and thrombus or recanalization with perivascular fibrosis, depending on the stage of disease. (3) Intermittent claudication typically begins in the arch of the foot and progresses to the calf; instep claudication is not typical of atherosclerotic occlusive disease.

Rest pain and diminished sensation from ischemic neuropathy are present in over 70% of patients at presentation. (4) Proximal pulses are normal, whereas distal pulses are absent. Digital disease is often asymmetric: Not all of the toes are affected to the same degree. The affected digits may be pale, cyanotic, erythematous, or gangrenous. (5) Ulcers are present in 75% of patients and are typically located at the nail margins. (6) The disease is never confined to one limb. Although not all limbs may be symptomatic, an abnormal Allen test or distal pulse examination can usually be demonstrated. (7) There is often a history of cold sensitivity or Raynaud's phenomenon. (8) The clinical course is usually episodic, with acute exacerbations followed by rather definite remissions. By contrast, steadily progressive symptoms are typical in atherosclerotic occlusive disease.

Atherosclerotic occlusive disease, emboli, and autoimmune disorders are included in the differential diagnosis. Several angiographic findings can be helpful in making these distinctions. Buerger's disease involves the distal arteries and spares the proximal arteries; it is segmental in appearance, with skip areas of disease; there is no vascular calcification; and extensive collateralization via tortuous "corkscrew" vessels is typical. Peripheral ultrasound or angiography and echocardiography are helpful in excluding an embolic source. Blood tests (complete blood count, coagulation studies, sedimentation rate, antinuclear antibody, lupus anticoagulant, rheumatoid factor, anticentromere antibody, and antiphospholipid antibody) are obtained to rule out vasculitis, lupus erythematosus, scleroderma, rheumatoid vascular disease, or a hypercoagulable state due to antiphospholipid antibody syndrome. A careful history is generally sufficient to exclude other rare disorders that may mimic Buerger's disease, such as ergotamine intoxication, cannabis arteritis, or small vessel occlusive disease secondary to the use of vibratory tools (hypothenar hammer syndrome).