Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Bal, Amanjit, Das, Ashim, Gupta, Dheeraj, Garg, Mandeep
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2014
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
_version_ 1782340281414189056
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
work_keys_str_mv AT balamanjit goodpasturessyndromeandpancaassociatedvasculitisinapatientofsilicosiderosisanunusualassociation
AT dasashim goodpasturessyndromeandpancaassociatedvasculitisinapatientofsilicosiderosisanunusualassociation
AT guptadheeraj goodpasturessyndromeandpancaassociatedvasculitisinapatientofsilicosiderosisanunusualassociation
AT gargmandeep goodpasturessyndromeandpancaassociatedvasculitisinapatientofsilicosiderosisanunusualassociation