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Kidney Week

Abstract: TH-PO388

Rare Presentation of Primary Sjögren Syndrome, Hypokalemic Paralysis: A Case Report

Session Information

Category: Fluid, Electrolytes, and Acid-Base Disorders

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

Authors

  • Anjum, Nosheen, KRL Hospital, Islamabad, Islamabad, Pakistan
  • Nabi, Zahid, KRL Hospital, Islamabad, Islamabad, Pakistan
Introduction

Sjogren's Syndrome is a multisystem autoimmune disorder characterized by chronic inflammation of exocrine glands, sicca syndrome. Among extra glandular manifestations renal involvement occurs as tubulointerstitial nephritis (TIN) and glomerular damage. Distal renal tubular acidosis (dRTA) is the most common presentation of TIN. However, dRTA may rarely be severe enough to present as hypokalemic paralysis. Also, Sjogren’s syndrome presenting for the first time with hypokalemic paralysis secondary to dRTA without other glandular manifestations is also very rare. Our patient had severe hypokalemic paralysis secondary to dRTA as the first manifestation of primary Sjogren’s syndrome.

Case Description

Methodology: We report a case of 45 years old lady, recently diagnosed as depressive illness with no other pre-morbid, who presented to us with acute onset of severe weakness in all four limbs for last 4 days. There was no other complaint. Clinical examination revealed hyporeflexia in four limbs with no other positive findings. Laboratory workup revealed severe hypokalemia, normal anion gap metabolic acidosis, alkaline urine and strongly positive serological markers of Sjogren’s syndrome, (anti-RO, anti-LA, ANA). Results: We treated that patient by intravascular replacement of potassium, oral, alkali, replacement and corticosteroids. Patient showed an excellent response to this treatment. She will be followed up for the recurrence of tubular dysfunction and other systemic manifestations of Sjogren’s syndrome.

Discussion

This report highlights that Sjogren's syndrome is a rare but an important cause of hypokalemia and therefore should be considered in diagnosis of hypokalemia.