Hypokalaemic paralysis due to underlying distal renal tubular acidosis as the first presentation of primary Sjogren’s syndrome: a case report

Abstract

Sjögren syndrome is a systemic autoimmune disease that primarily affects the exocrine glands. A 36-year-old woman presented with sudden onset of bilateral lower limb weakness. She was noted to have hypokalaemia with a normal anion gap metabolic acidosis. Subsequent investigations revealed high ESR, positive ANA, absent dsDNA, normal C3, C4, positive anti-Ro and negative anti-La. Labial biopsy revealed a lymphocytic infiltrate and renal biopsy revealed tubulointerstitial nephritis. Sjogren’s syndrome rarely (less than 5%) presents for the first time as renal manifestations such as hypokalaemia related paralysis as a result of type I – distal renal tubular acidosis due to the underlying tubulointerstitial nephritis.