Paralysis Unveiling Autoimmunity: A Case of Hypokalemic Paralysis due to Distal Renal Tubular Acidosis as Presenting Manifestation of Sjogren’s Syndrome

Abstract

Sjogren’s syndrome is a chronic autoimmune disorder primarily affecting exocrine glands, but it can also present with extra-glandular manifestations, including renal and neurological complications. Distal renal tubular acidosis is a rare but significant renal manifestation of Sjogren’s syndrome, often asymptomatic but can lead to severe hypokalemia. We report a case of a 44-year-old woman who presented with acute flaccid quadriparesis due to severe hypokalemia. Initial evaluation revealed normal central nervous system imaging. Laboratory investigations revealed hyperchloremic normal anion gap metabolic acidosis, elevated urine potassium-creatinine ratio, and alkaline urine, leading to a diagnosis of distal renal tubular acidosis. Further workup demonstrated positivity for SS-A/Ro and SS-B/La antibodies, along with clinical features of dry eyes and dry mouth, confirming Sjogren’s syndrome as the underlying etiology. Patient was treated with intravenous potassium followed by long-term potassium citrate, sodium bicarbonate, and immunosuppressive therapy. With appropriate management, she showed complete resolution of symptoms and remained stable during follow-up. This case highlights the importance of recognizing renal involvement as an early or atypical presentation of Sjogren’s syndrome, particularly in patients without overt sicca symptoms, and emphasizes the need for heightened clinical suspicion for systemic autoimmune disorders in patients presenting with unexplained hypokalemic paralysis.