Nephrocalcinosis: a rare presenting manifestation of primary Sjögren’s syndrome

Abstract

Renal involvement in primary Sjögren’s syndrome (pSS) is not uncommon. Autoimmune tubulointerstitial disorders and distal renal tubular acidosis (dRTA) account for majority of the cases of renal involvement. While dRTA may precede the onset of sicca syndrome in pSS, nephrocalcinosis as a presenting manifestation of pSS is rare. Here, to emphasize the need for initiating investigations for pSS in any patient presenting with nephrocalcinosis due to dRTA, we report a 21-year-old woman presenting with nephrocalcinosis long before pSS was objectively diagnosed.

Keywords::

• Distal renal tubular acidosis (dRTA)
• Primary Sjögren’s syndrome (pSS)
• Nephrocalcinosis