Long-term prognosis of Sjögren’s syndrome with periodic tetraplegia and distal tubular acidosis, a case report

A 28-year-old woman was hospitalised with periodic quadriplegia after a common cold. Hypokalemia and acidosis were diagnosed. The anion gap was normal, but urinary potassium excretion was increased, and the patient was diagnosed with distal renal tubular acidosis. She also had severe dry eye and mouth symptoms, and an SS-A (Ro) antibody test was positive, indicating Sjögren's syndrome. A potassium derivative and sodium bicarbonate were started, and her symptoms improved. The only finding on kidney biopsy was significant fibrosis of the distal tubules. This case suggests a strong relationship between distal renal tubular acidosis findings and distal tubular damage. We report a valuable case in which renal function has been preserved for 20 years after diagnosis without administration of glucocorticoid, and only by electrolyte correction.