Document Detail

A case of infection-associated antiproteinase-3-negative cytoplasmic antineutrophil cytoplasmic antibody pauci-immune focal necrotizing glomerulonephritis.
MedLine Citation:
PMID:  20566570     Owner:  NLM     Status:  MEDLINE    
We present the case of a man with Gram-negative sepsis and exposure to oral silica who developed pauci-immune focal necrotizing glomerulonephritis (PI-FNGN) in the setting of a subacute polymicrobial central venous line (CVL) infection. He developed a cytoplasmic antineutrophil cytoplasmic autoantibody (C-ANCA) that was antiproteinase-3 (PR-3) and antimyeloperoxidase (MPO) antibody negative. We believe this is the first reported case of Gram-negative sepsis-associated PI-FNGN. Chronic silica exposure is a leading environmental risk factor in the development of ANCA vasculitis. Oral silica is a common pharmaceutical additive and its bioavailability is being recognized. Oral silica, therefore, may also be a risk for development of autoreactivity. The PI-FNGN resolved with antibiotic therapy alone. The C-ANCA titer declined as the PI-FNGN resolved. The case supports experimental and observational research that environmental exposures act as adjuvants for an immune response and also provide epigenetic triggers for autoreactivity. The C-ANCA was negative for PR-3, its major antigen. C-ANCA antigen specificity may depend on the pathogenesis of the underlying disease, potentially elicited by a cross-reaction of an antibody to foreign and self target antigen sequence homology or alternatively elicited by antigenic epitope spread.
Emmy K Bell; Sumant S Chugh; William J Cook
Publication Detail:
Type:  Case Reports; Journal Article; Research Support, N.I.H., Extramural     Date:  2010-06-21
Journal Detail:
Title:  Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association     Volume:  25     ISSN:  1460-2385     ISO Abbreviation:  Nephrol. Dial. Transplant.     Publication Date:  2010 Sep 
Date Detail:
Created Date:  2010-08-25     Completed Date:  2010-12-23     Revised Date:  2014-06-19    
Medline Journal Info:
Nlm Unique ID:  8706402     Medline TA:  Nephrol Dial Transplant     Country:  England    
Other Details:
Languages:  eng     Pagination:  3119-23     Citation Subset:  IM    
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MeSH Terms
Administration, Oral
Antibodies, Antineutrophil Cytoplasmic / immunology*
Autoantibodies / immunology*
Focal Infection / immunology*,  pathology
Glomerulonephritis / etiology*,  pathology*
Gram-Negative Bacterial Infections / immunology*,  pathology
Peptide Hydrolases / immunology
Peroxidase / immunology
Sepsis / immunology,  pathology
Silicon Dioxide / adverse effects
Vasculitis / complications,  immunology
Grant Support
Reg. No./Substance:
0/Antibodies, Antineutrophil Cytoplasmic; 0/Autoantibodies; 7631-86-9/Silicon Dioxide; EC; EC 3.4.-/Peptide Hydrolases

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