The Immunogenetics of Vasculitis

Vasculitides are a cluster of diseases defined by an immune attack targeting vessels of different sizes. While most types of vasculitis have an undetermined cause, progress has been achieved in the recent decade in elucidating the mechanisms that participate in the inflammatory damage of the blood vessel wall. Several studies have emphasized that genetic susceptibility is an important aspect of the pathogenesis of vasculitides. The most prominent genetic risk loci for vasculitides reside within the major histocompatibility complex region. This indicates that the immune system is a major contributor to the pathogenesis of this group of diseases. In this chapter, we provide an updated overview of the etiology and pathogenesis of these entities with an emphasis on the major insights gained from recent genetic studies in the highly studied types of vasculitides.