Vasculitis: pathofysiologische aspecten

Primary systemic vasculitis (PSV) is an inflammatory vascular condition which is classified based on the size of the affected blood vessel. Different underlying immunological pathophysiological mechanisms are involved in vasculitis with involvement of the non-specific innate and the specific adaptive immune system. More and more is known about the genetic background of PSV. In ANCA-associated vasculitis (AAV) the neutrophil uses the neutrophil extracellular “traps” (NET) along with the complement system to induce endothelial cell damage. Certain cytokines are prominent in some PSV forms, like interleukin (IL)-6 in giant cell arteriitis (GCA). Specific intracellular signal transduction “pathways” can be overactivated like the JAK/STAT pathway in Behçet's disease and in large vessel vasculitis (LVV).