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IgG4-Related Disease With Renal and Pulmonary Involvement
Immunoglobulin G4-related disease (IgG4-RD) is a rare immune-mediated disease affecting multiple organs and tissues. There is often the presence of elevated serum Ig4 subtype with histological evidence of lymphoplasmacytic infiltration, fibrosis, and phlebitis. The mainstay of treatment is steroids...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cureus
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8428951/ https://www.ncbi.nlm.nih.gov/pubmed/34522549 http://dx.doi.org/10.7759/cureus.17071 |
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author | Osuorji, Chinenye Master, Kiron Osuorji, Ikenna |
author_facet | Osuorji, Chinenye Master, Kiron Osuorji, Ikenna |
author_sort | Osuorji, Chinenye |
collection | PubMed |
description | Immunoglobulin G4-related disease (IgG4-RD) is a rare immune-mediated disease affecting multiple organs and tissues. There is often the presence of elevated serum Ig4 subtype with histological evidence of lymphoplasmacytic infiltration, fibrosis, and phlebitis. The mainstay of treatment is steroids therapy. We report the case of a 66-year-old man presenting with acute on chronic renal failure and pulmonary nodules seen on PET-CT scan. He also had elevated serum IgG4 subclass and histological features in keeping with IgG4-RD. He failed steroid therapy but responded subsequently to rituximab with complete resolution of his symptoms. |
format | Online Article Text |
id | pubmed-8428951 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Cureus |
record_format | MEDLINE/PubMed |
spelling | pubmed-84289512021-09-13 IgG4-Related Disease With Renal and Pulmonary Involvement Osuorji, Chinenye Master, Kiron Osuorji, Ikenna Cureus Nephrology Immunoglobulin G4-related disease (IgG4-RD) is a rare immune-mediated disease affecting multiple organs and tissues. There is often the presence of elevated serum Ig4 subtype with histological evidence of lymphoplasmacytic infiltration, fibrosis, and phlebitis. The mainstay of treatment is steroids therapy. We report the case of a 66-year-old man presenting with acute on chronic renal failure and pulmonary nodules seen on PET-CT scan. He also had elevated serum IgG4 subclass and histological features in keeping with IgG4-RD. He failed steroid therapy but responded subsequently to rituximab with complete resolution of his symptoms. Cureus 2021-08-10 /pmc/articles/PMC8428951/ /pubmed/34522549 http://dx.doi.org/10.7759/cureus.17071 Text en Copyright © 2021, Osuorji et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. |
spellingShingle | Nephrology Osuorji, Chinenye Master, Kiron Osuorji, Ikenna IgG4-Related Disease With Renal and Pulmonary Involvement |
title | IgG4-Related Disease With Renal and Pulmonary Involvement |
title_full | IgG4-Related Disease With Renal and Pulmonary Involvement |
title_fullStr | IgG4-Related Disease With Renal and Pulmonary Involvement |
title_full_unstemmed | IgG4-Related Disease With Renal and Pulmonary Involvement |
title_short | IgG4-Related Disease With Renal and Pulmonary Involvement |
title_sort | igg4-related disease with renal and pulmonary involvement |
topic | Nephrology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8428951/ https://www.ncbi.nlm.nih.gov/pubmed/34522549 http://dx.doi.org/10.7759/cureus.17071 |
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