Background: Primary Sjögren's syndrome is associated in 6 to 25% of cases with kidney involvement Most often it is a chronic interstitial nephritis, generally asymptomatic but sometimes responsible for distal tubular acidosis which can be complicated by severe hypokalemia.
Case report: A 48-year-old woman had hypokalemic quadriplegia, distal renal tubular acidosis and chronic interstitial nephritis at kidney biopsy. Primary Sjögren's syndrome was diagnosed.
Discussion: We discuss briefly the clinical, biological and immunological features of 18 similar cases reported in the literature.