Pauci-immune crescentic glomerulonephritis is usually a manifestation of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. However, some patients with pauci-immune crescentic glomerulonephritis lack ANCA. This Review compares the prevalence, clinical manifestations, histopathology, and outcomes of ANCA-negative pauci-immune crescentic glomerulonephritis with that of ANCA-positive disease. The authors also discuss the possible pathogenesis of ANCA-negative pauci-immune crescentic glomerulonephritis, paying particular attention to the mechanisms and role of neutrophil activation.
- Min Chen
- Cees G. M. Kallenberg
- Ming-Hui Zhao