Renal Tubular Acidosis in Patients with Primary Sjögren's Syndrome

• Published in: Electrolyte & blood pressure : E & BP Vol. 15; no. 1; pp. 17 - 22
• Main Authors: Jung, Su Woong, Park, Eun Ji, Kim, Jin Sug, Lee, Tae Won, Ihm, Chun Gyoo, Lee, Sang Ho, Moon, Ju-Young, Kim, Yang Gyun, Jeong, Kyung Hwan
• Format: Journal Article
• Language: English
• Published: Korea (South) The Korean Society of Electrolyte Metabolism 01.09.2017; 전해질고혈압연구회
• Subjects: Case Report; 의약학; Hypokalemia; Osteomalacia; Sjögren's syndrome; Renal tubular acidosis
• ISSN: 1738-5997; 2092-9935
• DOI: 10.5049/EBP.2017.15.1.17

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.