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Sjögren’s syndrome is a chronic inflammatory autoimmune disease characterized by exocrine gland involvement and marked lymphocytic infiltration. Numerous reports of patients with Sjögren’s syndrome have described kidney damage, mainly involving distal tubule dysfunction, severe renal calcification, kidney stones, and rickets. We herein describe a patient with primary Sjögren’s syndrome who developed type I renal tubular acidosis with hypokalemia as the first symptom. This case highlights the possibility that an underlying autoimmune disorder should be considered in a patient presenting with distal tubular acidosis or recurrent hypokalemic periodic paralysis because treatment of the primary disease improves the outcome.

Type I renal tubular acidosis caused by Sjögren’s syndrome with hypokalemia as the first symptom: a case report