Crescentic Glomerulonephritis: New Aspects of Pathogenesis

SummaryThis review provides a summary of recent advances in the understanding of crescentic glomerulonephritis, focusing on antineutrophil cytoplasm antibody (ANCA)-associated vasculitis and anti–glomerular basement membrane (anti-GBM) antibody disease. In ANCA-associated vasculitis (AAV), four main conceptual advances are discussed as follows: (1) evidence for the pathogenicity of ANCA, (2) molecular mimicry and the role of infection in AAV, (3) evidence for aberrant T-cell responses and T-cell regulation in AAV, and (4) advances in understanding of genetic predisposition to AAV. In relation to anti-GBM disease we discuss the following: (1) the nature of the Goodpasture autoantigens, (2) T-cell responses and regulation in anti-GBM disease, and (3) human leukocyte antigen and non–human leukocyte antigen genetic associations.