The role of autoimmunity in thromboangiitis obliterans (Buerger's disease)

Thromboangiitis obliterans (TAO), or Buerger's disease, is a nonatherosclerotic segmental vasculitis that affects the small- and medium-sized arteries and veins of the extremities and is strongly associated with tobacco exposure. The immunopathogenesis of TAO remains largely unknown. In the acute phase of the disease, macrophages and occasional giant cells are observed in the characteristic intraluminal thrombus with a relatively mild infiltration of CD4⁺ and CD8⁺ T cells and macrophages in the internal lamina. VCAM-1, ICAM-1, and E-selectin expression on the surface of vascular endothelial cells is increased. A variety of circulating autoreactive antibodies targeting endothelial cells and vessel wall components are associated with active disease. One recent report suggests that removal of circulating antibodies by immunoadsorption may decrease disease severity. TAO has been associated positively and negatively with various MHC class 1 and 2 genes; however, genetic testing is not currently used for clinical diagnosis or management. The possible links between tobacco exposure and loss of tolerance for vascular tissues, current management strategy for patients with TAO, and opportunities for translational science are discussed.