NEPHROGENIC DIABETIC INSIPIDUS AND OTHER DEFECTS OF RENAL TUBULAR FUNCTION IN SJOERGREN'S SYNDROME

Polyuria and polydipsia were the presenting symptoms in a young woman with Sjogren's syndrome. Tests for maximal reabsorption of water revealed a total inability of the renal tubules to abstract solute-free water during a sustained infusion of vasopressin. In addition to the renal concentrating defect, there were hyperchloremic acidosis with a defect in urinary acidification, increased clearance of phosphate and urate, and generalized aminoaciduria—a combination of findings compatible with the "adult Fanconi syndrome." The possible relationship of renal dysfunction in Sjogren's syndrome to an immunologic abnormality remains to be elucidated.