Primary Sjögren's syndrome with renal tubular acidosis

Case report of familial occurrence and evidence for an association of primary Sjögren's syndrome in the presence of renal tubular acidosis with HLA-DRW9

Abstract

The comparative studies of patients with primary Sjögren's syndrome between in the presence of renal tubular acidosis and in the absence of renal tubular acidosis were performed.
Nine out of 30 patients with primary Sjögren's syndrome had renal tubular acidosis.
HLA-DRW9 was significantly increased in frequency in the patients with primary Sjögren's syndrome with renal tubular acidosis (6/9=66.7%), as compared to 26.2%(115/439) among control Japanese and 14.3%(3/21) among patients with primary Sjögren's syndrome without renal tubular acidosis.
The possibility that primary Sjögren's syndrome with renal tubular acidosis is genetically different from the patients with primary Sjögren's syndrome without renal tubular acidosis is discussed.