Fulminant primary manifestations of Wegener’s granulomatosis might not be pauci-immune

Wegener’s granulomatosis is an ANCA-associated small vessel vasculitis. Because histologically immune complex deposits are frequently lacking, the term pauci-immune has been introduced for this subgroup. We report a patient with fulminant, severe PR3-ANCA-positive Wegener’s granulomatosis and multi-...

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Autores principales: Schönermarck, Ulf, Grahovac, Maja, Sárdy, Miklós, Dolch, Michael, Wollenberg, Andreas
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2010
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4421412/
https://www.ncbi.nlm.nih.gov/pubmed/25949469
http://dx.doi.org/10.1093/ndtplus/sfq145
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author Schönermarck, Ulf
Grahovac, Maja
Sárdy, Miklós
Dolch, Michael
Wollenberg, Andreas
author_facet Schönermarck, Ulf
Grahovac, Maja
Sárdy, Miklós
Dolch, Michael
Wollenberg, Andreas
author_sort Schönermarck, Ulf
collection PubMed
description Wegener’s granulomatosis is an ANCA-associated small vessel vasculitis. Because histologically immune complex deposits are frequently lacking, the term pauci-immune has been introduced for this subgroup. We report a patient with fulminant, severe PR3-ANCA-positive Wegener’s granulomatosis and multi-organ involvement (upper respiratory tract, lung, kidneys, skin and general symptoms), who showed pronounced immunoglobulin and complement deposits within the skin biopsy. Our observation supports the hypothesis that immune complex deposits may be under-recognized in early lesions of ANCA-associated Wegener’s granulomatosis.
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spelling pubmed-44214122015-05-06 Fulminant primary manifestations of Wegener’s granulomatosis might not be pauci-immune Schönermarck, Ulf Grahovac, Maja Sárdy, Miklós Dolch, Michael Wollenberg, Andreas NDT Plus Case Report Wegener’s granulomatosis is an ANCA-associated small vessel vasculitis. Because histologically immune complex deposits are frequently lacking, the term pauci-immune has been introduced for this subgroup. We report a patient with fulminant, severe PR3-ANCA-positive Wegener’s granulomatosis and multi-organ involvement (upper respiratory tract, lung, kidneys, skin and general symptoms), who showed pronounced immunoglobulin and complement deposits within the skin biopsy. Our observation supports the hypothesis that immune complex deposits may be under-recognized in early lesions of ANCA-associated Wegener’s granulomatosis. Oxford University Press 2010-12 2010-08-05 /pmc/articles/PMC4421412/ /pubmed/25949469 http://dx.doi.org/10.1093/ndtplus/sfq145 Text en © The Author 2010. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For permissions, please e-mail: journals.permissions@oxfordjournals.org http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Case Report
Schönermarck, Ulf
Grahovac, Maja
Sárdy, Miklós
Dolch, Michael
Wollenberg, Andreas
Fulminant primary manifestations of Wegener’s granulomatosis might not be pauci-immune
title Fulminant primary manifestations of Wegener’s granulomatosis might not be pauci-immune
title_full Fulminant primary manifestations of Wegener’s granulomatosis might not be pauci-immune
title_fullStr Fulminant primary manifestations of Wegener’s granulomatosis might not be pauci-immune
title_full_unstemmed Fulminant primary manifestations of Wegener’s granulomatosis might not be pauci-immune
title_short Fulminant primary manifestations of Wegener’s granulomatosis might not be pauci-immune
title_sort fulminant primary manifestations of wegener’s granulomatosis might not be pauci-immune
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4421412/
https://www.ncbi.nlm.nih.gov/pubmed/25949469
http://dx.doi.org/10.1093/ndtplus/sfq145
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