Electrolyte Blood Press.  2017 Sep;15(1):17-22. 10.5049/EBP.2017.15.1.17.

Renal Tubular Acidosis in Patients with Primary Sjögren's Syndrome

• Jung SW
• Park EJ
• Kim JS
• Lee TW
• Ihm CG
• Lee SH
• Moon JY
• Kim YG
• Jeong KH

• KMID: 2391008
• DOI: http://doi.org/10.5049/EBP.2017.15.1.17

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.

Keyword

Sjögren's syndrome; Osteomalacia; Renal tubular acidosis; Hypokalemia

MeSH Terms

Acid-Base Equilibrium
Acidosis
Acidosis, Renal Tubular*
Autoantibodies
Diagnosis
Exocrine Glands
Fanconi Syndrome
Female
Femoral Fractures
Glomerulonephritis
Humans
Hypokalemia
Kidney
Muscle Weakness
Nephritis, Interstitial
Osteomalacia
Paralysis
Saliva
Tears
Autoantibodies

Figure

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  Fig. 1 Mild interstitial inflammation with tubular injury (PAS stain, original magnification ×200)

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