| Pathogenesis of PR3-ANCA associated vasculitis. | |
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MedLine Citation:
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PMID: 18162369 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Wegener's Granulomatosis (WG) is closely associated with antineutrophil cytoplasmic autoantibodies (ANCA), particularly those directed to proteinase 3 (PR3). ANCA directed to myeloperoxidase (MPO) are associated with microscopic polyangiitis (MPA) and the Churg Strauss syndrome. PR3-ANCA associated vasculitis differs from MPO-ANCA associated vasculitis particularly in the occurrence of granulomatous inflammation and more widespread disease in the former condition. Studies in experimental animals strongly suggest a pathogenic role for MPO-ANCA, but this is less clear for PR3-ANCA. Here, we review pathogenic pathways in PR3-ANCA associated vasculitis focussing on unique patterns of PR3 expression on the neutrophil membrane in WG, as surface expression of PR3 is a prerequisite for stimulation by PR3-ANCA; (PR3-specific) cellular immunity in WG; and the role of Staphylococcus aureus carriage in this disease. These factors, together, may explain, at least in part, differences in clinical expression between MPO-ANCA and PR3-ANCA associated vasculitis. |
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Authors:
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C G M Kallenberg |
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Publication Detail:
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Type: Journal Article; Review Date: 2007-12-26 |
Journal Detail:
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Title: Journal of autoimmunity Volume: 30 ISSN: 0896-8411 ISO Abbreviation: J. Autoimmun. Publication Date: 2008 Feb-Mar |
Date Detail:
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Created Date: 2008-01-15 Completed Date: 2008-04-11 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8812164 Medline TA: J Autoimmun Country: England |
Other Details:
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Languages: eng Pagination: 29-36 Citation Subset: IM |
Affiliation:
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Department of Rheumatology and Clinical Immunology, University Medical Center Groningen, University of Groningen, P.O. Box 30.001, 9700 RB Groningen, The Netherlands. c.g.m.kallenberg@int.umcg.nl |
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Antibodies, Antineutrophil Cytoplasmic / immunology*, metabolism Churg-Strauss Syndrome / immunology Humans Immunity, Cellular Myeloblastin / immunology*, metabolism Neutrophil Activation Neutrophils / enzymology, immunology*, metabolism Peroxidase / immunology*, metabolism Staphylococcus aureus / immunology Vasculitis / immunology*, physiopathology Wegener Granulomatosis / immunology |
| Chemical | |
Reg. No./Substance:
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0/Antibodies, Antineutrophil Cytoplasmic; EC 1.11.1.7/Peroxidase; EC 3.4.21.76/Myeloblastin |
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