Journal de vascularite

The Pathogenesis of Antineutrophil Antibody Associated Vasculitis: Environmental and Genetic Considerations

Abstract

Paul A Gatenby

The review seeks to summarise the current ideas in regard to the pathogenesis of ANCA-associated vasculitis and to examine in detail how epidemiological and genetic factors fit with the modern paradigm. The recent literature has been reviewed. The AASV appear to involve initiation by both T cells and B cells followed by a neutrophil dominated inflammatory phase in which ANCA may actually be involved. The alternative complement pathway may play a role. The genetic background is reviewed with genes identified that potentially encode proteins that are involved in the regulation of the immune system, other genes may be involved in the control of the inflammatory phase. With regard to environmental factors the two that stand out are a latitude gradient, presumably vitamin D and silica an agent known to be associated with both autoantibody production and autoimmune disease. A model which includes these factors is outlined.