Abstract: SA-PO0594
Life-Threatening Hypokalemia as the Initial Presentation of Distal Renal Tubular Acidosis-Associated Sjögren Syndrome
Session Information
- Fluid, Electrolyte, and Acid-Base Disorders: Case Reports - 2
October 24, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Fluid, Electrolytes, and Acid-Base Disorders
- 1102 Fluid, Electrolyte, and Acid-Base Disorders: Clinical
Authors
- Sharara, Jana, Cleveland Clinic, Cleveland, Ohio, United States
- Bindhu, Shwetha, Cleveland Clinic, Cleveland, Ohio, United States
- Alhaddad, Juliano, Cleveland Clinic, Cleveland, Ohio, United States
- Cavanaugh, Corey J., Cleveland Clinic, Cleveland, Ohio, United States
Introduction
Distal renal tubular acidosis (dRTA) is an uncommon but important renal manifestation of autoimmune diseases, most notably Sjögren’s syndrome. It results from impaired distal hydrogen ion secretion, leading to non–anion gap metabolic acidosis, hypokalemia, and an inappropriately elevated urine pH. Although hypokalemia is typical, cases with critical significance are rare. We present a case report describing a life-threatening presentation of dRTA as the initial manifestation of an underlying Sjögren’s syndrome.
Case Description
A 33-year-old woman presented with acute generalized weakness and syncope without known preceding illness, medication use, or toxin exposure. Laboratory workup revealed profound hypokalemia (<1.5 mmol/L), metabolic acidosis (bicarbonate 11 mmol/L, venous pH 7.2), and EKG findings of bradycardia with markedly prolonged QTc of 634. She was admitted to the ICU for aggressive potassium and bicarbonate replacement, and cardiac monitoring. Upon discussion, she was reported some chronic intermittent symptoms of fatigue, myalgias, polydipsia, and dry mouth, ongoing for months. Further workup to identify causes of electrolyte abnormalities revealed alkaline urine pH (7.5), positive urine anion gap (17.1), and renal potassium wasting, confirming distal RTA. Workup included screening for toxins and autoimmune diseases. Blood work was negative for lead, mercury, cadmium and toluene. Endocrine disorders and monoclonal gammopathy were also ruled out. Autoimmune testing showed high-titer ANA and strongly positive anti-SSA antibodies (>8 AI), supporting Sjögren’s syndrome. With treatment, electrolytes and QTc normalized, and muscle strength fully recovered. She was discharged on chronic potassium supplementation with close follow-up in nephrology and rheumatology clinics.
Discussion
This case highlights a rare but critical presentation of dRTA with life-threatening hypokalemia as a first presentation for Sjögren’s syndrome. Notably, classic Sjögren’s features were subtle, limited to intermittent xerostomia, which delayed the diagnosis. The combination of non–anion gap metabolic acidosis, high urine pH, and potassium wasting is key to diagnosing dRTA, while autoimmune serologies help establish the etiology. Clinicians should maintain suspicion for autoimmune disease in unexplained hypokalemia, even without overt rheumatologic symptoms.