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Rare case of atypical crescentic glomerulonephritis and interstitial lung disease with negative anti-GBM antibody and positive anti-MPO antibody

A 70-year-old man presented with 1 month of haematuria and mild right-sided flank pain with no other symptoms. Diagnostic workup included serum studies which showed the presence of antimyeloperoxidase antibodies, a kidney biopsy which demonstrated necrotising crescentic glomerulonephritis with linea...

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Autores principales: Hanna, Alexander, Ross, Jenny, Heitor, Fernanda
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BMJ Publishing Group 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6678005/
https://www.ncbi.nlm.nih.gov/pubmed/31371277
http://dx.doi.org/10.1136/bcr-2019-229256
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author Hanna, Alexander
Ross, Jenny
Heitor, Fernanda
author_facet Hanna, Alexander
Ross, Jenny
Heitor, Fernanda
author_sort Hanna, Alexander
collection PubMed
description A 70-year-old man presented with 1 month of haematuria and mild right-sided flank pain with no other symptoms. Diagnostic workup included serum studies which showed the presence of antimyeloperoxidase antibodies, a kidney biopsy which demonstrated necrotising crescentic glomerulonephritis with linear immunofluorescence of the basement membrane, and electron microscopy which exhibited thickening of the glomerular basement membrane. Incidentally, the patient was discovered to have a latent hepatitis B infection, which complicated immunosuppressive therapy. He was treated with a course of plasmapheresis and methylprednisolone, followed by entecavir for hepatitis B prophylaxis, and finally by rituximab. This case of glomerulonephritis was notable for its resemblance to the better known Goodpasture’s disease. Typically, Goodpasture’s syndrome exists on a spectrum from seronegative disease to double-positive disease that presents with both anti–glomerular basement membrane (anti-GBM) and cytoplasmic-antineutrophil cytoplasmic antibodies/antiproteinase 3 antibodies (c-ANCA/anti-PR3). However, this patient’s glomerulonephritis was unique because he presented negative for anti-GBM antibodies and positive for perinuclear-antineutrophil cytoplasmic antibodies/antimyeloperoxidase antibodies (p-ANCA/anti-MPO).
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spelling pubmed-66780052019-08-16 Rare case of atypical crescentic glomerulonephritis and interstitial lung disease with negative anti-GBM antibody and positive anti-MPO antibody Hanna, Alexander Ross, Jenny Heitor, Fernanda BMJ Case Rep Rare Disease A 70-year-old man presented with 1 month of haematuria and mild right-sided flank pain with no other symptoms. Diagnostic workup included serum studies which showed the presence of antimyeloperoxidase antibodies, a kidney biopsy which demonstrated necrotising crescentic glomerulonephritis with linear immunofluorescence of the basement membrane, and electron microscopy which exhibited thickening of the glomerular basement membrane. Incidentally, the patient was discovered to have a latent hepatitis B infection, which complicated immunosuppressive therapy. He was treated with a course of plasmapheresis and methylprednisolone, followed by entecavir for hepatitis B prophylaxis, and finally by rituximab. This case of glomerulonephritis was notable for its resemblance to the better known Goodpasture’s disease. Typically, Goodpasture’s syndrome exists on a spectrum from seronegative disease to double-positive disease that presents with both anti–glomerular basement membrane (anti-GBM) and cytoplasmic-antineutrophil cytoplasmic antibodies/antiproteinase 3 antibodies (c-ANCA/anti-PR3). However, this patient’s glomerulonephritis was unique because he presented negative for anti-GBM antibodies and positive for perinuclear-antineutrophil cytoplasmic antibodies/antimyeloperoxidase antibodies (p-ANCA/anti-MPO). BMJ Publishing Group 2019-08-01 /pmc/articles/PMC6678005/ /pubmed/31371277 http://dx.doi.org/10.1136/bcr-2019-229256 Text en © BMJ Publishing Group Limited 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.
spellingShingle Rare Disease
Hanna, Alexander
Ross, Jenny
Heitor, Fernanda
Rare case of atypical crescentic glomerulonephritis and interstitial lung disease with negative anti-GBM antibody and positive anti-MPO antibody
title Rare case of atypical crescentic glomerulonephritis and interstitial lung disease with negative anti-GBM antibody and positive anti-MPO antibody
title_full Rare case of atypical crescentic glomerulonephritis and interstitial lung disease with negative anti-GBM antibody and positive anti-MPO antibody
title_fullStr Rare case of atypical crescentic glomerulonephritis and interstitial lung disease with negative anti-GBM antibody and positive anti-MPO antibody
title_full_unstemmed Rare case of atypical crescentic glomerulonephritis and interstitial lung disease with negative anti-GBM antibody and positive anti-MPO antibody
title_short Rare case of atypical crescentic glomerulonephritis and interstitial lung disease with negative anti-GBM antibody and positive anti-MPO antibody
title_sort rare case of atypical crescentic glomerulonephritis and interstitial lung disease with negative anti-gbm antibody and positive anti-mpo antibody
topic Rare Disease
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6678005/
https://www.ncbi.nlm.nih.gov/pubmed/31371277
http://dx.doi.org/10.1136/bcr-2019-229256
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