Systemic vasculitides constitute a heterogeneous group of overlapping disea
ses characterized by blood vessel inflammation and necrosis. Although the c
auses of most forms of vasculitis are unknown, epidemiological studies have
implicated geographic, genetic, and environmental factors. Ethnicity, vari
ous genes such as those of the Major Histocompatibility Complex, gender, an
d environmental factors appear to account for differences in the incidence
of these syndromes. In this article, the authors focus on the incidence of
these conditions in different parts of the world and on the influence of en
vironmental and genetic factors.