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Buerger’s Disease (Thromboangiitis Obliterans)

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Systemic Vasculitides: Current Status and Perspectives

Abstract

Buerger’s disease (also known as thromboangiitis obliterans) is a non-atherosclerotic, inflammatory, segmental peripheral vascular occlusive disease that typically affects young male smokers. The patients typically present with ischemic symptoms caused by stenosis or occlusion of the distal small arteries. Superficial thrombophlebitis often occurs, which is migratory and frequently correlates with disease activity. The disease escalates usually at the age of 30–40 years and thereafter symptoms diminish. This disease had been more frequently reported in the Mediterranean, Middle East, and Far East Asia, but the number of patients is decreasing.

There are two characteristic histopathological findings. One is that inflammatory infiltrating cells are well recognized, predominantly in the thrombi and the intima. The other is that the internal elastic lamina and all layers of the vessel wall structures are well preserved. Buerger’s disease is distinguishable from atherosclerosis and other vasculitides by this characteristic preserved elastic lamina. There are several clinical diagnostic criteria for Buerger’s disease, which mostly require a compatible history, supportive physical findings, diagnostic vascular abnormalities on imaging studies, and current or past smoking.

The etiology of Buerger’s disease still remains to be elucidated and no therapeutic guidelines exist. However, smoking clearly associates with its exacerbation and remission. Absolute smoking cessation is the one and only definitive therapy for Buerger’s disease.

The fate of ischemic limb in Buerger’s disease is not so poor compared to that in atherosclerotic disease, provided that the patients maintain smoking cessation. As for the life expectancy, long-term survival is generally considered not to be affected by Buerger’s disease, due to the rare involvement of cerebral, coronary, and visceral arteries.

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Correspondence to Kimihio Komori .

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Sugimoto, M., Komori, K. (2016). Buerger’s Disease (Thromboangiitis Obliterans). In: Dammacco, F., Ribatti, D., Vacca, A. (eds) Systemic Vasculitides: Current Status and Perspectives. Springer, Cham. https://doi.org/10.1007/978-3-319-40136-2_31

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