Abstract
A 62-year-old woman with Sjögren's syndrome, distal renal tubular acidosis and hypokalemic muscle paralysis is described. The sicca syndrome was nearly subclinical and went unrecognized for several years. The main and first manifestation to be expressed was that of hypokalemic muscle paralysis secondary to renal tubular acidosis. In the last decade several reports have appeared indicating that renal tubular acidosis is associated with Sjögren's syndrome. The data in this report support the view that adult onset distal renal tubular acidosis is often a disorder of an autoimmune disease, frequently that of Sjögren's syndrome. The complications to renal tubular acidosis such as hypokalemic muscle paralysis or chronic muscle weakness, nephrolithiasis, and osteomalacia can be avoided if the diagnosis of renal tubular acidosis is made and corrective alkali therapy is maintained.