- Active tobacco use, typically moderate to heavy.
- Severe digital ischemia without evidence of internal organ involvement.
- Angiography reveals segmental involvement of medium-sized arteries, with abrupt vascular cut-offs and corkscrew collaterals.
- The major vessel involvement occurs at the levels of the ankle and wrist.
In Buerger disease, also called thromboangiitis obliterans, the classic patient is a young male smoker. The mean age of onset is approximately 40 years, but the disease can occur in teenagers as well as in the elderly. Although the patients described initially were men, the disease may afflict women as well, probably in direct proportion to the number of women in any particular society who smoke. The precise mechanism underlying the relationship between Buerger disease and cigarette smoking is unknown; autoimmune reactions to constituents of tobacco have been postulated. Cases may present several years after the start of smoking, but Buerger disease does not occur in the absence of ongoing tobacco exposure.
There are four keys to the diagnosis of Buerger disease: (1) Recognition of clinical findings compatible with that condition; namely, digital ischemia without involvement of other organs. (2) Identification of the typical pattern of vascular involvement by angiography. (3) Exclusion of diseases that may mimic Buerger disease (Table 41–1). (4) Confirmation that the major risk factor, ongoing tobacco exposure, is present.
Table 41–1. Differential Diagnosis of Buerger Disease. |Favorite Table|Download (.pdf)
Table 41–1. Differential Diagnosis of Buerger Disease.
- Cardiovascular conditions
- Cardiogenic emboli (eg, infective endocarditis)
- Systemic disorders associated with autoimmunity
- Systemic lupus erythematosus
- Antiphospholipid antibody syndrome
- Systemic sclerosis (particularly limited scleroderma, or CREST syndrome)
- Mixed connective tissue disease
- Systemic vasculitides
- Rheumatoid vasculitis
- Polyarteritis nodosa
- Granulomatosis with polyangiitis (formerly Wegener granulomatosis)
- Microscopic polyangiitis
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
Because of difficulty in accessing medium-sized vessels for biopsy, the diagnosis is rarely confirmed by biopsy. The exceptions to this rule are superficial thrombophlebitis, which seldom comes to medical attention, and amputation specimens, by which time medical attention is (at least in some senses) too late. When biopsy is possible, acute Buerger disease is characterized by a highly inflammatory thrombus, composed of a variety of cell types: lymphocytes, neutrophils, giant cells, and occasional microabscesses. Inflammation is typically more intense within the clot itself than within the walls of affected blood vessels. Fibrinoid necrosis, a hallmark of most systemic vasculitides, is absent in Buerger disease.
A major hallmark of Buerger disease is its confinement to the extremities. The initial symptoms may be nonspecific pains in the calf, foot, or toes. The progression of thrombosis and vasculitis can lead to horrific pain in the digits and limbs and ultimately to gangrene and tissue loss, through either autoamputation or elective amputation. For unknown reasons, however, other ...