Recurrent Hypokalemic Periodic Paralysis Unmasks Sjogren Syndrome without Sicca Symptoms

J Coll Physicians Surg Pak. 2015 Apr:25 Suppl 1:S36-8.

Abstract

Hypokalemic Periodic Paralysis (HPP) may occur as a rare complication of Sjogren Syndrome (SS) and Renal Tubular Acidosis (RTA). A 64-year male patient came with HPP, and was later diagnosed with distal RTA. The patient, who had no xerostomia and xerophthalmia, was diagnosed with primary SS from serologic and histologic findings of minor salivary gland biopsy. The patient recovered after potassium replacement therapy. Renal biopsy was also performed and revealed evidence of tubulointerstitial nephritis. Corticosteroids were administered and there was no recurrence of HPP during a 4-year follow-up period. The case highlights the significance of acute hypokalemia management in emergency department as it can unmask SS even if the SS is not associated with sicca symptoms. Hypokalemic paralysis associated with normal anion gap metabolic acidosis should prompt toward the diagnosis of SS.

Publication types

  • Case Reports

MeSH terms

  • Acidosis, Renal Tubular / diagnosis*
  • Acidosis, Renal Tubular / drug therapy
  • Acidosis, Renal Tubular / etiology
  • Antirheumatic Agents / therapeutic use
  • Biopsy
  • Cyclophosphamide / therapeutic use
  • Humans
  • Hypokalemic Periodic Paralysis / diagnosis
  • Hypokalemic Periodic Paralysis / drug therapy
  • Hypokalemic Periodic Paralysis / etiology*
  • Male
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Nephritis, Interstitial / complications
  • Nephritis, Interstitial / pathology
  • Potassium / administration & dosage
  • Sjogren's Syndrome / complications
  • Sjogren's Syndrome / diagnosis*
  • Sjogren's Syndrome / drug therapy
  • Treatment Outcome

Substances

  • Antirheumatic Agents
  • Cyclophosphamide
  • Potassium
  • Methylprednisolone