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03 June 2017 : Case report  USA

A Case of Kidney Involvement in Primary Sjögren’s Syndrome

Unusual clinical course, Challenging differential diagnosis, Diagnostic / therapeutic accidents, Educational Purpose (only if useful for a systematic review or synthesis)

Farid Arman1E, Hania Shakeri2F, Niloofar Nobakht1F, Anjay Rastogi1E, Mohammad Kamgar1E*

DOI: 10.12659/AJCR.903476

Am J Case Rep 2017; 18:622-626

Abstract

BACKGROUND: Sjögren’s syndrome is an autoimmune disorder caused by the infiltration of monocytes in epithelial glandular and extra-glandular tissues. Hallmark presentations include mouth and eye dryness. Although renal involvement is uncommon in primary Sjögren’s syndrome (pSS), patients may experience renal tubular acidosis type I (RTA I), tubulointerstitial nephritis, diabetes insipidus (DI), nephrolithiasis, and Fanconi syndrome. However, it is atypical to see more than 1 of these manifestations in a single patient.

CASE REPORT: We present the case of a 24-year-old woman with polyuria and polydipsia, who was initially diagnosed with nephrogenic diabetes insipidus. She also had chronic hypokalemia and nephrolithiasis. Based on clinical presentation and work up, she was diagnosed with pSS and treated accordingly.

CONCLUSIONS: This was a pSS patient with tubulointerstitial nephritis, diabetes insipidus, renal tubular acidosis, hypokalemia, and nephrolithiasis, who was receiving symptomatic treatment for diabetes insipidus. Diagnosis and treatment of pSS led to significant improvement in systemic and renal presentations of the patient. pSS should be considered as one of the differential diagnoses in patients with diabetes insipidus and renal tubular acidosis.

Keywords: Acidosis, Renal Tubular, Diabetes insipidus, Sjogren's Syndrome

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923