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Kidney Week

Abstract: TH-PO387

Unveiling the Enigma: Hypokalemic Paralysis in Sjögren-Associated Renal Tubular Acidosis

Session Information

Category: Fluid, Electrolytes, and Acid-Base Disorders

  • 1102 Fluid, Electrolyte, and Acid-Base Disorders: Clinical

Authors

  • Pathak, Shrirang Deepak, All India Institute of Medical Science - Bhopal, Bhopal, Madhya Pradesh, India
  • Kulkarni, Avani, All India Institute of Medical Science - Bhopal, Bhopal, Madhya Pradesh, India
  • Ingle, Vaibhav, All India Institute of Medical Science - Bhopal, Bhopal, Madhya Pradesh, India
Introduction

Hypokalemic Paralysis (HP) is a rare life-threatening disorder presenting as muscle weakness and low serum potassium levels. This case series focuses on two female patients with Sjögren's syndrome-associated renal tubular acidosis (SS-RTA) who developed HP. SS-RTA is a form of distal renal tubular acidosis where the kidneys are unable to adequately acidify urine and reabsorb bicarbonate, resulting in an acid-base imbalance. The SS-RTA is thought to occur due to inflammation caused by Sjögren's syndrome-associated autoantibodies. Although distal renal tubular acidosis causes hypokalemia, HP is rare. The important differential diagnoses include Hypokalemic Periodic Paralysis and Thyrotoxic Periodic Paralysis. The cases illustrate the association between SS-RTA and HP.

Case Description


Case 1: A 26-year-old female with intellectual impairment presented with vomiting, weakness, altered sensorium, and hypercapnic respiratory failure requiring intubation and mechanical ventilation. Investigations confirmed hypokalemia, normal anion gap metabolic acidosis (NAGMA), and SS-RTA. Treatment included intravenous (IV) potassium and sodium bicarbonate. Follow-up was done on an outpatient basis for correction of hypokalemia and NAGMA.
Case 2: A 47-year-old female had progressive limb weakness, slurred speech, reduced muscle power and absent reflexes. On investigations low serum potassium and positive serology for Primary Sjögren syndrome (PSS) were found, and renal biopsy findings of Membranoproliferative glomerulonephritis with chronic tubulointerstitial inflammation. Treatment involved IV potassium and IV fluids. She was readmitted for recurrence of hypokalemia.

Discussion

Hypokalemic Paralysis is characterized by muscle weakness and low serum potassium levels. SS-RTA leads to hypokalemia, triggering HP. Low magnesium and potassium levels and high creatine kinase levels are risk factors for severe paralysis in HP. The diagnosis is based on clinical, laboratory, and imaging findings. Management involves correcting acidosis, normalizing potassium, and addressing the underlying cause. Recognizing SS-RTA as a possible cause of hypokalemia in periodic paralysis, especially in Sjögren's syndrome, is essential. Early diagnosis and treatment are crucial for better outcomes. Further research is needed for delineating genotypes and phenotypes of PSS predisposing to HP.