Renal tubular acidosis without interstitial nephritis in Sjögren’s syndrome: a case report and review of the literature

BMC Nephrology

Case Report

Miki Takata¹  Shintaro Hamada²  Takaaki Sugihara²  Marie Yamamoto²  Tomoaki Takata²  Takuji Iyama²  Kentaro Yamada²  Hajime Isomoto²  Yukari Mae² 

[1] Department of Respiratory Medicine and Rheumatology Graduate School of Medicine, Tottori University Hospital, 683-8504, Yonago, Tottori, Japan;Division of Gastroenterology and Nephrology, Tottori University Faculty of Medicine, 683-8504, Yonago, Tottori, Japan;

关键词: Hypokalemia;    Renal tubular acidosis;    SJÖGREN’S syndrome;    H-ATPase;    Interstitial nephritis;   

DOI  :  10.1186/s12882-023-03290-3

 received in 2022-02-10, accepted in 2023-08-03,  发布年份 2023

来源: Springer

【 摘 要 】

BackgroundRenal 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 presentationA 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.ConclusionsThis 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.

【 授权许可】

CC BY   
© BioMed Central Ltd., part of Springer Nature 2023

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