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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...

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Autores principales: Inoue, Reiko, Fujigaki, Yoshihide, Kobayashi, Kana, Tamura, Yoshifuru, Ota, Tatsuru, Shibata, Shigeru, Ishida, Tsuyoshi, Kondo, Fukuo, Yamaguchi, Yutaka, Uchida, Shunya
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2017
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.
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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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