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IgA nephropathy to proliferative glomerulonephritis with monoclonal IgAκ deposits: a pattern switch

We report the case of a 31-year-old male who presented with repeated episodes of nephritic-nephrotic syndrome in concomitance with infection. IgA was diagnosed and was initially responsive to treatment with immunosuppressors but further disease flare did not respond to treatment. Based on three cons...

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Autores principales: Li, Jin-pu, Du, Ya-ting, Guo, Chuan, Rao, Xiang-rong, Li, Shen
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer International Publishing 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10638190/
https://www.ncbi.nlm.nih.gov/pubmed/36913081
http://dx.doi.org/10.1007/s40620-023-01583-2
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author Li, Jin-pu
Du, Ya-ting
Guo, Chuan
Rao, Xiang-rong
Li, Shen
author_facet Li, Jin-pu
Du, Ya-ting
Guo, Chuan
Rao, Xiang-rong
Li, Shen
author_sort Li, Jin-pu
collection PubMed
description We report the case of a 31-year-old male who presented with repeated episodes of nephritic-nephrotic syndrome in concomitance with infection. IgA was diagnosed and was initially responsive to treatment with immunosuppressors but further disease flare did not respond to treatment. Based on three consecutive renal biopsies over 8 years, a pattern switch from endocapillary proliferative IgA nephropathy to membranous proliferative glomerulonephritis with monoclonal IgAκ deposits was observed. Bortezomib-dexamethasone combination therapy finally led to a favorable renal response. This case provides new insights into the pathophysiological mechanisms of proliferative glomerulonephritis with monoclonal immunoglobin deposits (PGNMID), highlighting the importance of repeat renal biopsies and routine evaluation of monoclonal immunoglobin deposits in proliferative glomerulonephritis with refractory nephrotic syndrome.
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spelling pubmed-106381902023-11-14 IgA nephropathy to proliferative glomerulonephritis with monoclonal IgAκ deposits: a pattern switch Li, Jin-pu Du, Ya-ting Guo, Chuan Rao, Xiang-rong Li, Shen J Nephrol Case Report We report the case of a 31-year-old male who presented with repeated episodes of nephritic-nephrotic syndrome in concomitance with infection. IgA was diagnosed and was initially responsive to treatment with immunosuppressors but further disease flare did not respond to treatment. Based on three consecutive renal biopsies over 8 years, a pattern switch from endocapillary proliferative IgA nephropathy to membranous proliferative glomerulonephritis with monoclonal IgAκ deposits was observed. Bortezomib-dexamethasone combination therapy finally led to a favorable renal response. This case provides new insights into the pathophysiological mechanisms of proliferative glomerulonephritis with monoclonal immunoglobin deposits (PGNMID), highlighting the importance of repeat renal biopsies and routine evaluation of monoclonal immunoglobin deposits in proliferative glomerulonephritis with refractory nephrotic syndrome. Springer International Publishing 2023-03-13 2023 /pmc/articles/PMC10638190/ /pubmed/36913081 http://dx.doi.org/10.1007/s40620-023-01583-2 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Case Report
Li, Jin-pu
Du, Ya-ting
Guo, Chuan
Rao, Xiang-rong
Li, Shen
IgA nephropathy to proliferative glomerulonephritis with monoclonal IgAκ deposits: a pattern switch
title IgA nephropathy to proliferative glomerulonephritis with monoclonal IgAκ deposits: a pattern switch
title_full IgA nephropathy to proliferative glomerulonephritis with monoclonal IgAκ deposits: a pattern switch
title_fullStr IgA nephropathy to proliferative glomerulonephritis with monoclonal IgAκ deposits: a pattern switch
title_full_unstemmed IgA nephropathy to proliferative glomerulonephritis with monoclonal IgAκ deposits: a pattern switch
title_short IgA nephropathy to proliferative glomerulonephritis with monoclonal IgAκ deposits: a pattern switch
title_sort iga nephropathy to proliferative glomerulonephritis with monoclonal igaκ deposits: a pattern switch
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10638190/
https://www.ncbi.nlm.nih.gov/pubmed/36913081
http://dx.doi.org/10.1007/s40620-023-01583-2
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