Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
Decreased Renal Expression of H+-ATPase and Pendrin in a Patient with Distal Renal Tubular Acidosis Associated with Sjögren's Syndrome
Ha Yeon KimSung Sun KimEun Hui BaeSeong Kwon MaSoo Wan Kim
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2015 Volume 54 Issue 22 Pages 2899-2904


A 31-year-old woman with no significant past medical or family history was admitted with complaints of general weakness. Laboratory tests revealed: serum potassium 3.0 mEq/L, arterial blood pH 7.28, serum bicarbonate 17.8 mEq/L and urinary pH 7.0. Double-labeling confocal fluorescence microscopy using H+-ATPase and pendrin antibodies demonstrated a decreased expression of these proteins in the patient's renal collecting duct compared to normal controls. Anti-Sjögren's-syndrome-related antigen A (Anti-Ro/SS-A) and anti-Sjögren's syndrome type B (anti-La/SS-B) antibodies were strongly positive with very high titers, consistent with Sjögren's syndrome. We present a case of distal renal tubular acidosis-associated Sjögren's syndrome with a defect in H+-ATPase and pendrin in the renal collecting duct.

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© 2015 by The Japanese Society of Internal Medicine
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