ABSTRACT

Vasculitides are chronic inflammatory diseases in which blood vessel walls are targeted by an immune insult. For the last decades, necrosis of the vessel wall as well as thrombotic occlusion of the vascular lumen have been considered to be the major pathological pathways. The standard paradigm for the immunopathogenesis of inflammatory vasculopathies has centered around the assumption that endothelial injury is the leading event followed by the formation of inflammatory cell infiltrates within and around the vessel wall. It is now clear that this paradigm is oversimplified. From advances in cell and molecular biology, immunology, and molecular genetics, several new key concepts have emerged that, when integrated with the clinical syndromes of vasculitis, will facilitate an explosion of new knowledge and improved patient care.