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Case Reports
. 2017 Sep;15(1):17-22.
doi: 10.5049/EBP.2017.15.1.17. Epub 2017 Sep 30.

Renal Tubular Acidosis in Patients with Primary Sjögren's Syndrome

Su Woong Jung  1 , Eun Ji Park  2 , Jin Sug Kim  1 , Tae Won Lee  2 , Chun Gyoo Ihm  2 , Sang Ho Lee  3 , Ju-Young Moon  3 , Yang Gyun Kim  3 , Kyung Hwan Jeong  2
Affiliations
Case Reports

Renal Tubular Acidosis in Patients with Primary Sjögren's Syndrome

Su Woong Jung et al. Electrolyte Blood Press. 2017 Sep.

Abstract

Primary Sjögren's syndrome (pSS) is characterized by lymphocytic infiltration of the exocrine glands resulting in decreased saliva and tear production. It uncommonly involves the kidneys in various forms, including tubulointerstitial nephritis, renal tubular acidosis, Fanconi syndrome, and rarely glomerulonephritis. Its clinical symptoms include muscle weakness, periodic paralysis, and bone pain due to metabolic acidosis and electrolyte imbalance. Herein, we describe the cases of two women with pSS whose presenting symptoms involve the kidneys. They had hypokalemia and normal anion gap metabolic acidosis due to distal renal tubular acidosis and positive anti-SS-A and anti-SS-B autoantibodies. Since one of them experienced femoral fracture due to osteomalacia secondary to renal tubular acidosis, an earlier diagnosis of pSS is important in preventing serious complications.

Keywords: Hypokalemia; Osteomalacia; Renal tubular acidosis; Sjögren's syndrome.

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Conflict of interest statement

Conflict of Interest: The authors declare no relevant financial interests.

Figures

Fig. 1
Fig. 1. Mild interstitial inflammation with tubular injury (PAS stain, original magnification ×200)
See this image and copyright information in PMC

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