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Clinical Presentation of Tubulointerstitial Nephritis Caused by Amyloid Light-chain Amyloidosis in a Patient with Sjögren's Syndrome
We report a 70-year-old woman with Sjögren's syndrome who had severe renal dysfunction with mild proteinuria and elevated urinary low-molecular-weight proteins. Based on these clinical presentations, interstitial nephritis due to Sjögren's syndrome was strongly suspected. Unexpectedly, ren...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Japanese Society of Internal Medicine
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5364195/ https://www.ncbi.nlm.nih.gov/pubmed/28202864 |
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author | Inoue, Reiko Fujigaki, Yoshihide Kobayashi, Kana Tamura, Yoshifuru Ota, Tatsuru Shibata, Shigeru Ishida, Tsuyoshi Kondo, Fukuo Yamaguchi, Yutaka Uchida, Shunya |
author_facet | Inoue, Reiko Fujigaki, Yoshihide Kobayashi, Kana Tamura, Yoshifuru Ota, Tatsuru Shibata, Shigeru Ishida, Tsuyoshi Kondo, Fukuo Yamaguchi, Yutaka Uchida, Shunya |
author_sort | Inoue, Reiko |
collection | PubMed |
description | We report a 70-year-old woman with Sjögren's syndrome who had severe renal dysfunction with mild proteinuria and elevated urinary low-molecular-weight proteins. Based on these clinical presentations, interstitial nephritis due to Sjögren's syndrome was strongly suspected. Unexpectedly, renal pathology revealed amyloid light-chain (AL) lambda-type depositions predominantly in the vasculatures with severe tubulointerstitial damage. Concentrated urine immunofixation was positive for Bence Jones lambda-type monoclonal proteins. Given the involvement in other organs, systemic AL amyloidosis was diagnosed. The patient underwent chemotherapy, but hemodialysis was ultimately instituted. It should be remembered that renal amyloidosis occurs as a clinical presentation of interstitial nephritis. |
format | Online Article Text |
id | pubmed-5364195 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | The Japanese Society of Internal Medicine |
record_format | MEDLINE/PubMed |
spelling | pubmed-53641952017-03-24 Clinical Presentation of Tubulointerstitial Nephritis Caused by Amyloid Light-chain Amyloidosis in a Patient with Sjögren's Syndrome Inoue, Reiko Fujigaki, Yoshihide Kobayashi, Kana Tamura, Yoshifuru Ota, Tatsuru Shibata, Shigeru Ishida, Tsuyoshi Kondo, Fukuo Yamaguchi, Yutaka Uchida, Shunya Intern Med Case Report We report a 70-year-old woman with Sjögren's syndrome who had severe renal dysfunction with mild proteinuria and elevated urinary low-molecular-weight proteins. Based on these clinical presentations, interstitial nephritis due to Sjögren's syndrome was strongly suspected. Unexpectedly, renal pathology revealed amyloid light-chain (AL) lambda-type depositions predominantly in the vasculatures with severe tubulointerstitial damage. Concentrated urine immunofixation was positive for Bence Jones lambda-type monoclonal proteins. Given the involvement in other organs, systemic AL amyloidosis was diagnosed. The patient underwent chemotherapy, but hemodialysis was ultimately instituted. It should be remembered that renal amyloidosis occurs as a clinical presentation of interstitial nephritis. The Japanese Society of Internal Medicine 2017-02-15 /pmc/articles/PMC5364195/ /pubmed/28202864 Text en https://creativecommons.org/licenses/by-nc-nd/4.0/ The Internal Medicine is an Open Access article distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Case Report Inoue, Reiko Fujigaki, Yoshihide Kobayashi, Kana Tamura, Yoshifuru Ota, Tatsuru Shibata, Shigeru Ishida, Tsuyoshi Kondo, Fukuo Yamaguchi, Yutaka Uchida, Shunya Clinical Presentation of Tubulointerstitial Nephritis Caused by Amyloid Light-chain Amyloidosis in a Patient with Sjögren's Syndrome |
title | Clinical Presentation of Tubulointerstitial Nephritis Caused by Amyloid Light-chain Amyloidosis in a Patient with Sjögren's Syndrome |
title_full | Clinical Presentation of Tubulointerstitial Nephritis Caused by Amyloid Light-chain Amyloidosis in a Patient with Sjögren's Syndrome |
title_fullStr | Clinical Presentation of Tubulointerstitial Nephritis Caused by Amyloid Light-chain Amyloidosis in a Patient with Sjögren's Syndrome |
title_full_unstemmed | Clinical Presentation of Tubulointerstitial Nephritis Caused by Amyloid Light-chain Amyloidosis in a Patient with Sjögren's Syndrome |
title_short | Clinical Presentation of Tubulointerstitial Nephritis Caused by Amyloid Light-chain Amyloidosis in a Patient with Sjögren's Syndrome |
title_sort | clinical presentation of tubulointerstitial nephritis caused by amyloid light-chain amyloidosis in a patient with sjögren's syndrome |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5364195/ https://www.ncbi.nlm.nih.gov/pubmed/28202864 |
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