Renal tubular acidosis without interstitial nephritis in Sjögren’s syndrome: a case report and review of the literature
Abstract Background Renal tubular acidosis is the principal clinical feature associated with tubulointerstitial nephritis in patients with primary Sjögren’s syndrome. Renal tubular dysfunction due to interstitial nephritis has been considered the underlying pathophysiology connecting renal tubular a...
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BMC
2023-08-01
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Online Access: | https://doi.org/10.1186/s12882-023-03290-3 |
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author | Shintaro Hamada Tomoaki Takata Kentaro Yamada Marie Yamamoto Yukari Mae Takuji Iyama Takaaki Sugihara Miki Takata Hajime Isomoto |
author_facet | Shintaro Hamada Tomoaki Takata Kentaro Yamada Marie Yamamoto Yukari Mae Takuji Iyama Takaaki Sugihara Miki Takata Hajime Isomoto |
author_sort | Shintaro Hamada |
collection | DOAJ |
description | Abstract Background Renal tubular acidosis is the principal clinical feature associated with tubulointerstitial nephritis in patients with primary Sjögren’s syndrome. Renal tubular dysfunction due to interstitial nephritis has been considered the underlying pathophysiology connecting renal tubular acidosis and primary Sjögren’s syndrome. However, the detailed mechanisms underlying the pathophysiology of renal tubular acidosis in primary Sjögren’s syndrome is not fully understood. Case presentation A 30-year-old woman was admitted with complaints of weakness in the extremities. The patient was hospitalized thirteen years earlier for similar issues and was diagnosed with hypokalemic paralysis due to distal renal tubular acidosis with primary Sjögren’s syndrome. This diagnosis was based on a positive Schirmer's test. Besides, anti-Sjögren’s syndrome-related antigen A was also detected. Laboratory tests indicated distal RTA; however, a renal biopsy showed no obvious interstitial nephritis. Laboratory tests conducted during the second admission indicated distal renal tubular acidosis. Therefore, a renal biopsy was performed again, which revealed interstitial nephritis. Histological analysis of acid–base transporters revealed the absence of vacuolar type H+-ATPases in the collecting duct. The vacuolar type H+-ATPase was also absent in the past renal biopsy, suggesting that the alteration in acid–base transporters is independent of interstitial nephritis. Conclusions This case study demonstrates that vacuolar-type H+-ATPases are associated with distal renal tubular acidosis, and distal renal tubular acidosis precedes interstitial nephritis in patients with primary Sjögren’s syndrome. |
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spelling | doaj.art-15b7aa3c8e774b6cb2594eb406a7e4472023-11-19T12:39:23ZengBMCBMC Nephrology1471-23692023-08-012411610.1186/s12882-023-03290-3Renal tubular acidosis without interstitial nephritis in Sjögren’s syndrome: a case report and review of the literatureShintaro Hamada0Tomoaki Takata1Kentaro Yamada2Marie Yamamoto3Yukari Mae4Takuji Iyama5Takaaki Sugihara6Miki Takata7Hajime Isomoto8Division of Gastroenterology and Nephrology, Tottori University Faculty of MedicineDivision of Gastroenterology and Nephrology, Tottori University Faculty of MedicineDivision of Gastroenterology and Nephrology, Tottori University Faculty of MedicineDivision of Gastroenterology and Nephrology, Tottori University Faculty of MedicineDivision of Gastroenterology and Nephrology, Tottori University Faculty of MedicineDivision of Gastroenterology and Nephrology, Tottori University Faculty of MedicineDivision of Gastroenterology and Nephrology, Tottori University Faculty of MedicineDepartment of Respiratory Medicine and Rheumatology Graduate School of Medicine, Tottori University HospitalDivision of Gastroenterology and Nephrology, Tottori University Faculty of MedicineAbstract Background Renal tubular acidosis is the principal clinical feature associated with tubulointerstitial nephritis in patients with primary Sjögren’s syndrome. Renal tubular dysfunction due to interstitial nephritis has been considered the underlying pathophysiology connecting renal tubular acidosis and primary Sjögren’s syndrome. However, the detailed mechanisms underlying the pathophysiology of renal tubular acidosis in primary Sjögren’s syndrome is not fully understood. Case presentation A 30-year-old woman was admitted with complaints of weakness in the extremities. The patient was hospitalized thirteen years earlier for similar issues and was diagnosed with hypokalemic paralysis due to distal renal tubular acidosis with primary Sjögren’s syndrome. This diagnosis was based on a positive Schirmer's test. Besides, anti-Sjögren’s syndrome-related antigen A was also detected. Laboratory tests indicated distal RTA; however, a renal biopsy showed no obvious interstitial nephritis. Laboratory tests conducted during the second admission indicated distal renal tubular acidosis. Therefore, a renal biopsy was performed again, which revealed interstitial nephritis. Histological analysis of acid–base transporters revealed the absence of vacuolar type H+-ATPases in the collecting duct. The vacuolar type H+-ATPase was also absent in the past renal biopsy, suggesting that the alteration in acid–base transporters is independent of interstitial nephritis. Conclusions This case study demonstrates that vacuolar-type H+-ATPases are associated with distal renal tubular acidosis, and distal renal tubular acidosis precedes interstitial nephritis in patients with primary Sjögren’s syndrome.https://doi.org/10.1186/s12882-023-03290-3HypokalemiaRenal tubular acidosisSJÖGREN’S syndromeH+-ATPaseInterstitial nephritis |
spellingShingle | Shintaro Hamada Tomoaki Takata Kentaro Yamada Marie Yamamoto Yukari Mae Takuji Iyama Takaaki Sugihara Miki Takata Hajime Isomoto Renal tubular acidosis without interstitial nephritis in Sjögren’s syndrome: a case report and review of the literature BMC Nephrology Hypokalemia Renal tubular acidosis SJÖGREN’S syndrome H+-ATPase Interstitial nephritis |
title | Renal tubular acidosis without interstitial nephritis in Sjögren’s syndrome: a case report and review of the literature |
title_full | Renal tubular acidosis without interstitial nephritis in Sjögren’s syndrome: a case report and review of the literature |
title_fullStr | Renal tubular acidosis without interstitial nephritis in Sjögren’s syndrome: a case report and review of the literature |
title_full_unstemmed | Renal tubular acidosis without interstitial nephritis in Sjögren’s syndrome: a case report and review of the literature |
title_short | Renal tubular acidosis without interstitial nephritis in Sjögren’s syndrome: a case report and review of the literature |
title_sort | renal tubular acidosis without interstitial nephritis in sjogren s syndrome a case report and review of the literature |
topic | Hypokalemia Renal tubular acidosis SJÖGREN’S syndrome H+-ATPase Interstitial nephritis |
url | https://doi.org/10.1186/s12882-023-03290-3 |
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