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- Title
Shining a LAMP on pauci-immune focal segmental glomerulonephritis.
- Authors
Salama, Alan D.; Pusey, Charles D.
- Abstract
Anti-neutrophil cytoplasm antibody (ANCA)–associated vasculitis frequently presents with renal involvement manifested by a focal segmental necrotizing glomerulonephritis, which is typically pauci-immune. Although considerable insight has been gained regarding potential mechanisms of organ damage, researchers have remained relatively ignorant of the initiating factors breaking immune tolerance. A recent report has provided evidence that molecular mimicry may be critical, with immune responsiveness toward a bacterial fimbrial protein inducing a cross-reactive autoimmune response toward lysosomal-associated membrane protein-2 (LAMP-2). Use of an experimental model demonstrates that this response generates ANCA and provokes pulmonary-renal disease, reminiscent of human ANCA–associated vasculitis. Greater understanding of the immune mechanisms underlying the development of ANCA should lead to more focused approaches to the treatment of small-vessel vasculitis.Kidney International (2009) 76, 15–17; doi:10.1038/ki.2009.123; published online 22 April 2009
- Subjects
GLOMERULONEPHRITIS; KIDNEY glomerulus diseases; IMMUNOGLOBULINS; MEMBRANE proteins; VASCULITIS; NEPHROLOGY
- Publication
Kidney International, 2009, Vol 76, Issue 1, p15
- ISSN
0085-2538
- Publication type
Article
- DOI
10.1038/ki.2009.123