Hypokalemic Quadriparesis as the Initial Presentation of Primary Sjögren's Syndrome With Distal Renal Tubular Acidosis: A Case Report.

Primary Sjögren's syndrome (PSS) typically presents with sicca symptoms, while renal involvement, such as distal renal tubular acidosis (dRTA), is less common. Hypokalemic paralysis as the initial manifestation of PSS is rare. We report a 52-year-old woman who presented with acute flaccid quadriparesis due to severe hypokalemia and normal anion gap metabolic acidosis consistent with dRTA. Autoimmune workup revealed ANA, anti-Ro/SSA, and anti-La/SSB positivity with an abnormal Schirmer's test, establishing the diagnosis of PSS despite the absence of dryness symptoms. The patient improved rapidly with potassium and bicarbonate replacement. This case underscores the importance of considering PSS in patients with unexplained hypokalemic paralysis and metabolic acidosis, even in the absence of sicca features, as timely recognition prevents life-threatening complications.