| A case of infection-associated antiproteinase-3-negative cytoplasmic antineutrophil cytoplasmic antibody pauci-immune focal necrotizing glomerulonephritis. | |
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MedLine Citation:
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PMID: 20566570 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Emmy K Bell; Sumant S Chugh; William J Cook |
Publication Detail:
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Type: Case Reports; Journal Article; Research Support, N.I.H., Extramural Date: 2010-06-21 |
Journal Detail:
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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:
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Created Date: 2010-08-25 Completed Date: 2010-12-23 Revised Date: 2011-09-13 |
Medline Journal Info:
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Nlm Unique ID: 8706402 Medline TA: Nephrol Dial Transplant Country: England |
Other Details:
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Languages: eng Pagination: 3119-23 Citation Subset: IM |
Affiliation:
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Division of Nephrology, Department of Medicine, University of Alabama at Birmingham, Birmingham, AL, USA. ekbell@uab.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Administration, Oral Adult Antibodies, Antineutrophil Cytoplasmic / immunology* Autoantibodies / immunology* Focal Infection / immunology*, pathology Glomerulonephritis / etiology*, pathology* Gram-Negative Bacterial Infections / immunology*, pathology Humans Male Peptide Hydrolases / immunology Peroxidase / immunology Sepsis / immunology, pathology Silicon Dioxide / adverse effects Vasculitis / complications, immunology |
| Grant Support | |
ID/Acronym/Agency:
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5R01DK077073/DK/NIDDK NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Antibodies, Antineutrophil Cytoplasmic; 0/Autoantibodies; 7631-86-9/Silicon Dioxide; EC 1.11.1.7/Peroxidase; EC 3.4.-/Peptide Hydrolases |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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