Immunogenetic aspects of ANCA-associated vasculitides

The objective of this issue is to review the genetic bases and immunogenetic variations of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV), with particular emphasis on major histocompatibility complex class I and II molecules, the polymorphisms in the intercellular adhesion molecule-1 gene, tumor necrosis factor genes, alpha-1-anti-trypsin (alpha 1AT) and Fc gamma receptor (Fc gamma R) genes. Although the aetiologies of the various AAV are unknown, the available genetic epidemiological data support the importance of genetic factors in susceptibility to these diseases. Moreover, there is considerable reason to believe that immunologic mechanisms contribute to the pathogenesis of AAV. The data presented here indicate that, in addition to immunospecific genes, such as HLA alleles, genes determining the level of the immune response (i.e. cytokines, adhesion molecules, protease inhibitors, Fc gamma R) may contribute to AAV.