Distal Renal Tubular Acidosis in a Patient with Sjogren's Syndrome: Case Eeport and Literature Review

摘要

Distal renal tubular acidosis (dRTA) is associated with inadequate urinary acidification by the distal nephron. dRTA can present as either an inherited or acquired condition such as autoimmune disorders. The reported prevalence of renal involvement in Sjogren's syndrome (SS) patients varies widely, ranging from 2 to 67 percent. A defect in distal acidification occurs in up to 25 percent of patients with Sjogren's syndrome. We report a 32-year-old woman who presented with progressive flaccid quadriparesis. Laboratory investigations showed severe hypokalemia with renal K~+ wasting and hyperchloremic metabolic acidosis. A positive urine anion gap with alkaline urine and hypercalciuria with nephrolithiasis were consistent with the diagnosis of distal RTA. Further investigations revealed Sjogren's syndrome as the underlying cause of distal RTA.