Decreased Renal Expression of H+-ATPase and Pendrin in a Patient with Distal Renal Tubular Acidosis Associated with Sjogren's Syndrome

作者：Kim Ha Yeon; Kim Sung Sun; Bae Eun Hui; Ma Seong Kwon; Kim Soo Wan^*

来源：Internal Medicine, 2015, 54(22): 2899-2904.

DOI：10.2169/internalmedicine.54.4821

• hypokalemia
• renal tubular acidosis
• Sjogren's syndrome
• H+-ATPase
• pendrin

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-Sjogren's-syndrome-related antigen A (Anti-Ro/SS-A) and anti-Sjogren's syndrome type B (anti-La/SS-B) antibodies were strongly positive with very high titers, consistent with Sjogren's syndrome. We present a case of distal renal tubular acidosis-associated Sjogren's syndrome with a defect in H+-ATPase and pendrin in the renal collecting duct.

• 出版日期2015