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Goodpasture's Syndrome and p-ANCA Associated Vasculitis in a Patient of Silicosiderosis: An Unusual Association
Introduction. Goodpasture's syndrome is a rare clinical entity and is characterized by circulating autoantibodies which are principally directed against the glomerular/alveolar basement membrane. The etiology of Goodpasture's syndrome is still unknown. Lung involvement occurs as a result o...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Hindawi Publishing Corporation
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4202243/ https://www.ncbi.nlm.nih.gov/pubmed/25349763 http://dx.doi.org/10.1155/2014/398238 |
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author | Bal, Amanjit Das, Ashim Gupta, Dheeraj Garg, Mandeep |
author_facet | Bal, Amanjit Das, Ashim Gupta, Dheeraj Garg, Mandeep |
author_sort | Bal, Amanjit |
collection | PubMed |
description | Introduction. Goodpasture's syndrome is a rare clinical entity and is characterized by circulating autoantibodies which are principally directed against the glomerular/alveolar basement membrane. The etiology of Goodpasture's syndrome is still unknown. Lung involvement occurs as a result of lung injury and the exposure of new epitopes to the immune system. Recently, several studies have suggested the role of silica as one of etiological factors in ANCA associated vasculitis and glomerulonephritis. Materials and Methods. We present a case of a 40-year-old welder with silicosiderosis, who developed anti-GBM disease with p-ANCA positivity. Case Report. Patient presented to an emergency with gradually increasing breathlessness along with renal failure and died after short hospital stay. Autopsy pathology findings revealed crescentic glomerulonephritis with linear glomerular basement membrane antibody deposition, splenic vasculitis, pulmonary haemorrhage, and pulmonary silicosiderosis. Conclusion. This case reinforces the role of environmental triggers like exposure to silica, metal dust, and tobacco in pathogenesis of Goodpasture's syndrome and p-ANCA associated vasculitis. |
format | Online Article Text |
id | pubmed-4202243 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | Hindawi Publishing Corporation |
record_format | MEDLINE/PubMed |
spelling | pubmed-42022432014-10-27 Goodpasture's Syndrome and p-ANCA Associated Vasculitis in a Patient of Silicosiderosis: An Unusual Association Bal, Amanjit Das, Ashim Gupta, Dheeraj Garg, Mandeep Case Rep Pulmonol Case Report Introduction. Goodpasture's syndrome is a rare clinical entity and is characterized by circulating autoantibodies which are principally directed against the glomerular/alveolar basement membrane. The etiology of Goodpasture's syndrome is still unknown. Lung involvement occurs as a result of lung injury and the exposure of new epitopes to the immune system. Recently, several studies have suggested the role of silica as one of etiological factors in ANCA associated vasculitis and glomerulonephritis. Materials and Methods. We present a case of a 40-year-old welder with silicosiderosis, who developed anti-GBM disease with p-ANCA positivity. Case Report. Patient presented to an emergency with gradually increasing breathlessness along with renal failure and died after short hospital stay. Autopsy pathology findings revealed crescentic glomerulonephritis with linear glomerular basement membrane antibody deposition, splenic vasculitis, pulmonary haemorrhage, and pulmonary silicosiderosis. Conclusion. This case reinforces the role of environmental triggers like exposure to silica, metal dust, and tobacco in pathogenesis of Goodpasture's syndrome and p-ANCA associated vasculitis. Hindawi Publishing Corporation 2014 2014-10-02 /pmc/articles/PMC4202243/ /pubmed/25349763 http://dx.doi.org/10.1155/2014/398238 Text en Copyright © 2014 Amanjit Bal et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Bal, Amanjit Das, Ashim Gupta, Dheeraj Garg, Mandeep Goodpasture's Syndrome and p-ANCA Associated Vasculitis in a Patient of Silicosiderosis: An Unusual Association |
title | Goodpasture's Syndrome and p-ANCA Associated Vasculitis in a Patient of Silicosiderosis: An Unusual Association |
title_full | Goodpasture's Syndrome and p-ANCA Associated Vasculitis in a Patient of Silicosiderosis: An Unusual Association |
title_fullStr | Goodpasture's Syndrome and p-ANCA Associated Vasculitis in a Patient of Silicosiderosis: An Unusual Association |
title_full_unstemmed | Goodpasture's Syndrome and p-ANCA Associated Vasculitis in a Patient of Silicosiderosis: An Unusual Association |
title_short | Goodpasture's Syndrome and p-ANCA Associated Vasculitis in a Patient of Silicosiderosis: An Unusual Association |
title_sort | goodpasture's syndrome and p-anca associated vasculitis in a patient of silicosiderosis: an unusual association |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4202243/ https://www.ncbi.nlm.nih.gov/pubmed/25349763 http://dx.doi.org/10.1155/2014/398238 |
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