Cargando…

Goodpasture’s syndrome with absence of circulating anti-glomerular basement membrane antibodies: a case report

BACKGROUND: Goodpasture’s syndrome, a rare disease, is an organ-specific autoimmune disease mediated by anti-glomerular basement membrane antibodies. Its pathology is characterized by crescentic glomerulonephritis with linear immunofluorescent staining for immunoglobulin G on the glomerular basement...

Descripción completa

Detalles Bibliográficos
Autores principales: Fernandes, Rui, Freitas, Sara, Cunha, Pedro, Alves, Gloria, Cotter, Jorge
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4962374/
https://www.ncbi.nlm.nih.gov/pubmed/27459964
http://dx.doi.org/10.1186/s13256-016-0984-6
_version_ 1782444821969895424
author Fernandes, Rui
Freitas, Sara
Cunha, Pedro
Alves, Gloria
Cotter, Jorge
author_facet Fernandes, Rui
Freitas, Sara
Cunha, Pedro
Alves, Gloria
Cotter, Jorge
author_sort Fernandes, Rui
collection PubMed
description BACKGROUND: Goodpasture’s syndrome, a rare disease, is an organ-specific autoimmune disease mediated by anti-glomerular basement membrane antibodies. Its pathology is characterized by crescentic glomerulonephritis with linear immunofluorescent staining for immunoglobulin G on the glomerular basement membrane. Although rare, a few cases with absence of circulating anti-glomerular membrane antibodies have been described. CASE PRESENTATION: The objective of this clinical case report is to describe and discuss a case of a 27-year-old white man who was hospitalized with a 1-year history of weight loss and a 1-month history of hemoptysis, with aggravation  the day before, having developed dyspnea and cough in the previous 24 hours. An analytical study showed normocytic normochromic anemia with a hemoglobin level of 7.2 g/dL and leukocytosis with normal renal function and coagulation times. A blood transfusion was performed without complications. Chest computed tomography revealed a reticulonodular infiltrate of both lungs. Bronchoscopy showed no apparent lesions. Sputum cultures, rapid urine antigens for Legionella pneumophila and Streptococcus pneumoniae, studies for Influenza, virologic markers and serologic studies for autoimmunity were all negative. At the end of the tenth day his general state deteriorated with fatigue, hematuria, and in 3 days he developed aggravation of renal function with recurrent hemoptysis and anemia. Immunosuppression with daily prednisolone 1 g administered intravenously was initiated. An urgent bronchoscopy showed no lesions. A kidney biopsy showed fibrinoid necrosis and cellular crescents. Immunofluorescence revealed a linear immunoglobulin G deposition compatible with Goodpasture’s syndrome. Immunosuppressive therapy with daily cyclophosphamide 120 mg orally was added. Subsequently he was transferred to a referral center at which 21 sessions of plasmapheresis and four sessions of hemodialysis were performed with good response; he currently has no need of hemodialysis. CONCLUSIONS: The absence of circulating anti-glomerular basement membrane antibodies in Goodpasture’s syndrome adds complexity to the diagnosis creating an unusual setting in a rare disease. In our case a kidney biopsy was essential for diagnosis and clinical approach. Studies have shown that early aggressive therapy leads to an improved prognosis. Physicians should consider tissue diagnoses such as bronchoscopy and kidney biopsy in pulmonary renal syndrome.
format Online
Article
Text
id pubmed-4962374
institution National Center for Biotechnology Information
language English
publishDate 2016
publisher BioMed Central
record_format MEDLINE/PubMed
spelling pubmed-49623742016-07-28 Goodpasture’s syndrome with absence of circulating anti-glomerular basement membrane antibodies: a case report Fernandes, Rui Freitas, Sara Cunha, Pedro Alves, Gloria Cotter, Jorge J Med Case Rep Case Report BACKGROUND: Goodpasture’s syndrome, a rare disease, is an organ-specific autoimmune disease mediated by anti-glomerular basement membrane antibodies. Its pathology is characterized by crescentic glomerulonephritis with linear immunofluorescent staining for immunoglobulin G on the glomerular basement membrane. Although rare, a few cases with absence of circulating anti-glomerular membrane antibodies have been described. CASE PRESENTATION: The objective of this clinical case report is to describe and discuss a case of a 27-year-old white man who was hospitalized with a 1-year history of weight loss and a 1-month history of hemoptysis, with aggravation  the day before, having developed dyspnea and cough in the previous 24 hours. An analytical study showed normocytic normochromic anemia with a hemoglobin level of 7.2 g/dL and leukocytosis with normal renal function and coagulation times. A blood transfusion was performed without complications. Chest computed tomography revealed a reticulonodular infiltrate of both lungs. Bronchoscopy showed no apparent lesions. Sputum cultures, rapid urine antigens for Legionella pneumophila and Streptococcus pneumoniae, studies for Influenza, virologic markers and serologic studies for autoimmunity were all negative. At the end of the tenth day his general state deteriorated with fatigue, hematuria, and in 3 days he developed aggravation of renal function with recurrent hemoptysis and anemia. Immunosuppression with daily prednisolone 1 g administered intravenously was initiated. An urgent bronchoscopy showed no lesions. A kidney biopsy showed fibrinoid necrosis and cellular crescents. Immunofluorescence revealed a linear immunoglobulin G deposition compatible with Goodpasture’s syndrome. Immunosuppressive therapy with daily cyclophosphamide 120 mg orally was added. Subsequently he was transferred to a referral center at which 21 sessions of plasmapheresis and four sessions of hemodialysis were performed with good response; he currently has no need of hemodialysis. CONCLUSIONS: The absence of circulating anti-glomerular basement membrane antibodies in Goodpasture’s syndrome adds complexity to the diagnosis creating an unusual setting in a rare disease. In our case a kidney biopsy was essential for diagnosis and clinical approach. Studies have shown that early aggressive therapy leads to an improved prognosis. Physicians should consider tissue diagnoses such as bronchoscopy and kidney biopsy in pulmonary renal syndrome. BioMed Central 2016-07-27 /pmc/articles/PMC4962374/ /pubmed/27459964 http://dx.doi.org/10.1186/s13256-016-0984-6 Text en © The Author(s). 2016 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Fernandes, Rui
Freitas, Sara
Cunha, Pedro
Alves, Gloria
Cotter, Jorge
Goodpasture’s syndrome with absence of circulating anti-glomerular basement membrane antibodies: a case report
title Goodpasture’s syndrome with absence of circulating anti-glomerular basement membrane antibodies: a case report
title_full Goodpasture’s syndrome with absence of circulating anti-glomerular basement membrane antibodies: a case report
title_fullStr Goodpasture’s syndrome with absence of circulating anti-glomerular basement membrane antibodies: a case report
title_full_unstemmed Goodpasture’s syndrome with absence of circulating anti-glomerular basement membrane antibodies: a case report
title_short Goodpasture’s syndrome with absence of circulating anti-glomerular basement membrane antibodies: a case report
title_sort goodpasture’s syndrome with absence of circulating anti-glomerular basement membrane antibodies: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4962374/
https://www.ncbi.nlm.nih.gov/pubmed/27459964
http://dx.doi.org/10.1186/s13256-016-0984-6
work_keys_str_mv AT fernandesrui goodpasturessyndromewithabsenceofcirculatingantiglomerularbasementmembraneantibodiesacasereport
AT freitassara goodpasturessyndromewithabsenceofcirculatingantiglomerularbasementmembraneantibodiesacasereport
AT cunhapedro goodpasturessyndromewithabsenceofcirculatingantiglomerularbasementmembraneantibodiesacasereport
AT alvesgloria goodpasturessyndromewithabsenceofcirculatingantiglomerularbasementmembraneantibodiesacasereport
AT cotterjorge goodpasturessyndromewithabsenceofcirculatingantiglomerularbasementmembraneantibodiesacasereport