A Rare Renal Twist in Sjögren Syndrome: Type I RTA

Abstract

 Sjögren's syndrome (SS) is an autoimmune condition that causes chronic inflammatory and degenerative changes in exocrine glands and systemic organs. Rare in adolescents, it often goes undiagnosed due to absent xerostomia, xerophthalmia, or sicca symptoms. Adolescents may initially present with parotitis or systemic organ involvement. We report a 36-year-old woman with recurrent severe hypokalemic episodes since age 21, ultimately diagnosed with type I (distal) renal tubular acidosis (RTA) due to SS. Despite significant hypokalemic paralysis in her background, the diagnosis was delayed as distal RTA is rare in this age group. The diagnosis was confirmed following severe hypokalemia, non-anion gap metabolic acidosis, raised urine anion gap and pH, supported by autoimmune workup. She was successfully managed with potassium and alkali replacement therapy, which stabilized her condition. This case highlights diagnostic challenges of SS when initial symptoms deviate from typical exocrine manifestations