Buerger's disease (Thromboangiitis obliterans)
is a syndrome of occlusion
of small and medium sized arteries that occurs almost exclusively in heavy smokers. When Dr. Leo Buerger first described it in 1909, it was felt to be a problem that only affected young male smoker
s. However, as the number of women smokers has increased the past 90 years, so has the prevalence
of Buerger's disease in women, although it still favors men 3:1.
The symptoms tend to wax and wane in early disease and are often asymmetric, and onset can be gradual or sudden. Coldness, numbness and sensations of burning or tingling are frequent, and ulceration or necrosis of digits is not uncommon. Cramping of hands or feet with use and "postural color changes" where the extremities are white with elevation and red when dependent are other symptoms.
The disease can be associated with Raynaud's phenomenon, but it is distinguished from it by its more dire consequences, its less classic symptoms and the absolute relationship to smoking.
The relationship to cigarette smoking is one of the most consistent aspects of this disorder, and most patients show hypersensitivity to intradermally injected tobacco extracts. Several possibilities have been postulated for this association, including direct endothelial cell toxicity induced by or hypersensitivity to some tobacco products. There is an increased prevalence of the human leukocyte antigens-A9 and HLA-B5 in these patients, and the condition is far more common in Israel, Japan, and India than in the United States and Europe, all of which hints at genetic influences. The incidence is 13 cases per 10,000 people in the U.S.
Microscopically acute and chronic inflammation of the small arteries occurs in a sharply segmental pattern, accompanied by thrombosis of the lumen, which may undergo organization and recanalization. Characteristically the thrombus contains small microabscesses marked by a central focus of neutrophils surrounded by granulomatous inflammation.
Primary treatment consists of smoking cessation. Supportive treatment and antibiotics may be necessary for necrosis and gangrene. nifedipine, a calcium-channel blocker which helps dilate arterioles, has been used but is no substitute for stopping smoking.