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Buerger's disease

Orpha number ORPHA36258
Prevalence of rare diseases 1-5 / 10 000
Inheritance -
Age of onset Adulthood
ICD 10 code
  • I73.1
MIM number
Synonym(s) Thromboangiitis obliterans

Summary

Thromboangiitis obliterans or Buerger's disease is a segmental occlusive inflammatory condition of arteries and veins, with thrombosis and recanalization of the affected vessels. It is a nonatherosclerotic inflammatory disease affecting small and medium sized arteries and veins of upper and lower extremities. 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 prevalence of the disease in the general population in Japan was estimated at 5/100,000 persons in 1985. The clinical criteria edited by Olin in 2000 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 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 role of other treatments including vasodilating or anti-clotting drugs, surgical revascularization or sympathectomy in preventing amputation or treating pain, remains unclear. *Author: Dr P.E.T. Arkkila (April 2006)*. Reproduced from Thromboangiitis obliterans (Buerger's disease). Orphanet J Rare Dis. 2006;1:14.

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