Abstract: PUB266
A Rare Case of Sjogren Syndrome Associated with Acquired Gitelman Syndrome
Session Information
Category: Fluid, Electrolytes, and Acid-Base Disorders
- 1102 Fluid, Electrolyte, and Acid-Base Disorders: Clinical
Author
- Ramirez Botana, Leonardo R., LSU Health Shreveport, Shreveport, Louisiana, United States
Introduction
Acquired renal tubular disorder has been detected in various disease processes, specifically autoimmune diseases. Gitelman syndrome is an autosomal recessive disease characterized by hypokalemic metabolic alkalosis, hypomagnesemia, and hypocalciuria. This case reported an acquired Gitelman syndrome associated with Sjögren's syndrome.
Case Description
A 61-year-old female with past medical history of Sjögren's syndrome, bronchiectasis, hypothyroidism was admitted due to right hip pain after a fall. She has been presenting with muscle weakness and cramping of lower extremities for several weeks. On phhysical examination was found low blood pressure, right lower hip pain, decreased range of motion and externally rotate right lower extremity. Initial laboratory results was found elevated white blood cell count, severe hypokalemia and metabolic alkalosis, hypocalcemia and hypomagnesemia. Urine spot chloride was reported 26 mEq/L , greater than 20 mEq/L, having with this value a differential diagnostic with intermittent diuretic use, and urine calcium excretion and maganesium are low as well. Renal magnesium wasting and hypomagnesemia are found in Gitelman syndrome, as well as,spot urine calcium/creatinine ratio of less than 44 mg/g, as our patient. This urine labs are explained due to the sodium chloride cotransporter (NCC) in Gitelman syndrome are shed into the urine in nanovesicles called urine exosomes and reducing the NCC activity in urine exosomes has been described in patients with Gitelman syndrome
Discussion
Our patients who was found with an unexplained hypokalemia, metabolic alkalosis and persistently elevated urine chloride for a Gitelman syndrome. It was reviewed other more common etiologies to excluded, and her home medications side effects was revised. In other cases, that has been reported a phenotype resembling Gitelman syndrome that can result from autoimmune diseases, most commonly in patients with Sjögren's disease–associated interstitial nephritis, for that reason, renal biopsy and genetic studies were ordered and will be follow up the results.The diagnosis of acquired Gitelman syndrome in our cases was based upon the clinical and laboratory findings. The hip was surgical repair and treated her electrolyte abnormalities and hypovolemia. Oral supplementation with substantial doses of sodium, potassium chloride and magnesium salts was ordered as fisrt line of therapy