In this article, Jennette and Falk review the clinical and experimental evidence implicating antineutrophil cytoplasmic autoantibodies (ANCAs) in the pathogenesis of pauci-immune systemic necrotizing small-vessel vasculitis and glomerulonephritis. They describe the current model of ANCA-mediated vascular inflammation, involving ANCA-induced activation of primed neutrophils, and extend this theory to describe a scenario in which these antibodies could instigate extravascular granulomatosis.
- J. Charles Jennette
- Ronald J. Falk
