Thromboangiitis obliterans (Buerger's disease)

Perttu E T Arkkila
Orphanet Journal of Rare Diseases 2006 April 27, 1: 14
Thromboangiitis obliterans or Buerger's disease is a segmental occlusive inflammatory condition of arteries and veins, characterized by thrombosis and recanalization of the affected vessels. It is a non-atherosclerotic inflammatory disease affecting small and medium sized arteries and veins of upper and lower extremities. The clinical criteria include: age under 45 years; current or recent history of tobacco use; presence of distal-extremity ischemia indicated by claudication, pain at rest, ischemic ulcers or gangrenes and documented by non-invasive vascular testing; exclusion of autoimmune diseases, hypercoagulable states and diabetes mellitus; exclusion of a proximal source of emboli by echocardiography or arteriography; consistent arteriographic findings in the clinically involved and non-involved limbs. The disease is found worldwide, the prevalence among all patients with peripheral arterial disease ranges from values as low as 0.5 to 5.6% in Western Europe to values as high as 45 to 63% in India, 16 to 66% in Korea and Japan, and 80% among Ashkenazi Jews. The etiology of thromboangiitis obliterans is unknown, but use or exposure to tobacco is central to the initiation and progression of the disease. If the patient smokes, stopping completely is an essential first step of treatment. The effectiveness of other treatments including vasodilating or anti-clotting drugs, surgical revascularization or sympathectomy in preventing amputation or treating pain, remains to be determined.

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