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Abstract: SA-PO987

Simultaneous Renal Tubular Acidosis and Nephrogenic Diabetes Insipidus in a Patient with Sjogren’s Syndrome

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports


  • Hanouneh, Mohamad A., Johns Hopkins University, Baltimore, Maryland, United States
  • Menez, Steven, Johns Hopkins University, Baltimore, Maryland, United States
  • Monroy-Trujillo, Jose Manuel, Johns Hopkins University, Baltimore, Maryland, United States

There are several renal manifestations of Sjogren's Disease. Among them are distal and proximal renal tubular acidosis, diabetes insipidus, tubulointerstitial nephritis. However, it is unusual to find multiple manifestations simultaneously.


39 year old woman with medical history significant for anorexia nervosa (BMI 17 kg/m2) was transferred to psychiatric unit for management of eating disorder. She additionally had nausea, vomiting, polyuria and polydipsia. She complained of longstanding dry eyes and mouth. She described prolonged history of joint pain and recurrent nephrolithiasis.

She was only taking Sertraline 100 mg twice a day. Vitals were normal. Mucous membranes were significantly dry. She had significant digital clubbing. Cardiac, pulmonary, and neurological exams were normal. Urine output was 4-5 L/daily.

Workup revealed non-anion gap metabolic acidosis with hypokalemia (serum sodium 138 mmol/L, serum potassium 3.0 mmol/L, sodium HCO3 20 mmol/L, serum chloride 108 mmol/L). Serum creatinine was 1.1 mg/dL which was stable over the prior year. Urine anion gap had a positive value. (Urine sodium 20 mmol/L, urine potassium 10 mmol/L, urine chloride 21 mmol/L) and urine osmolality 130 mosm/kg. CT abdomen showed bilateral nephrocalcinosis with no hydronephrosis. She was diagnosed with distal renal tubular acidosis.

Regarding polyuria and polydipsia, diabetes insipidus was suspected given development of hypernatremia (her serum Na trended up to 145 mmol/L) and low urine osmolality (Urine Osmolality was 150 mosm/kg at that time). Patient received desmopressin with no significant change in urine volume and urine osmolality. Anti-nuclear antibodies (ANA) and Ro antibodies were strongly positive. Schirmer’s test confirmed the finding of dry eyes. She was diagnosed with Sjogren’s syndrome based on The American College of Rheumatology (ACR) criteria. Patient was treated symptomatically with eye drops, recommended to continue oral hydration driven by thirst and to have water available at all times. She was also started on hydroxychloroquine 200 mg PO daily. She did not tolerate potassium citrate given GI symptoms.


Distal RTA with nephrocalcinosis and nephrogenic DI are known renal manifestations of Sjogren ’s syndrome. However, it is unusual to find both manifestations simultaneously.