Abstract
Antibody-mediated glomerular disease is illustrated by antineutrophil cytoplasmic autoantibody-associated vascuLitis (AAV) and antiglomerular basement membrane (anti-GBM) disease. In both of these diseases, the autoantibodies play central roles in pathogenesis. Serum autoantibody concentrations are commonly used for diagnosis and to follow treatment effect. The factors leading to autoantibody formation are a combination of genetics and environmental exposures. Once the autoantibodies are formed, they eLicit disease by directly binding their respective epitopes, triggering neutrophil activation, complement deposition, and inflammatory cell recruitment. A rapidly progressive glomerulonephritis ensues that is potentially fatal if not diagnosed and treated in a timely manner. Animal models have served to significantly enhance our understanding of AAV and anti-GBM disease mechanisms. Further exploration of inflammatory disease mediators, potential disease triggers, and genetic risks is important to develop targeted treatments and to inform patients about potential risk factors.
Original language | English |
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Title of host publication | Pathobiology of Human Disease |
Subtitle of host publication | A Dynamic Encyclopedia of Disease Mechanisms |
Publisher | Elsevier Inc. |
Pages | 2802-2811 |
Number of pages | 10 |
ISBN (Electronic) | 9780123864567 |
ISBN (Print) | 9780123864574 |
DOIs | |
State | Published - Jan 1 2014 |
Keywords
- Antiglomerular basement membrane (anti-GBM)
- Antineutrophil cytoplasmic autoantibody (ANCA)
- Crescentic glomerulonephritis
- Glomerulonephritis
- Pauci-immune glomerulonephritis
- Rapidly progressive glomerulonephritis