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A rare inflammatory, non-necrotizing, non-atherosclerotic, occlusive vascular disease characterized by thrombosis and recanalization affecting small and medium sized arteries and veins of upper and lower extremities.
ORPHA:36258Classification level: Disorder
The prevalence is estimated at 1/10,000 in Europe but the disease is found worldwide.
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.
Management and treatment
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.
Article for general public
- Review article
- English (2006)