Nephrocalcinosis and hypokalemia in a patient with primary Sjogren's syndrome

Clinically significant renal involvement is uncommon in primary Sjogren's syndrome, amid which tubulointerstitial disorders, distal renal tubular acidosis (dRTA) particularly, account for the majority. Conversely, Sjogren's syndrome comprises at least half the patients presenting with renal tubular acidosis. While underlying dRTA itself is an important cause of nephrocalcinosis and urolithiasis, nephrocalcinosis is rarely a presenting feature of primary Sjogren's syndrome. I report a 41-year-old female contracting nephrocalcinosis and hypokalemia as complications of primary Sjogren's syndrome with dRTA, hereby to emphasize the importance of alkali therapy.