Distal Renal Tubular Acidosis in Sjögren's Syndrome: A Case Report

Abstract

Sjögren’s syndrome is an autoimmune lymphocytic infiltrative disease that leads to chronic inflammatory and degradatory changes to exocrine glands and extra-glandular systemic organs. It rarely affects children and adolescents. In cases where adolescents are affected, a paucity of sicca symptoms, xerostomia, and xerophthalmia often leads to a missed diagnosis. Consequently, the first presenting sign of Sjögren’s syndrome in adolescents may be heterogeneous, with varying clinical symptoms related to parotitis or systemic organ involvement. In this case report, we discuss a 19-year-old girl with distal renal tubular acidosis (RTA), who had experienced severe hypokalemic episodes since the age of 14 years; the patient was eventually diagnosed with Sjögren’s syndrome. She was managed and maintained on potassium and alkali repletion therapy.