Vol.23 No.1

Case Report

Renal involvement in primary Sjogren syndrome of childhood: case report and literature review

Authors

Radovan Bogdanović1,2 , Gordana Basta-Jovanović1 , Jovana Putnik2 , Nataša Stajić2 , Aleksandra Paripović2

  • Faculty of Medicine, University of Belgrade, 8 Dr Subotica Str, 11000, Belgrade, Serbia
  • Institute of Mother and Child Healthcare of Serbia “ Dr Vukan Čupić”, 6-8 Radoja Dakica Street, 11070, Belgrade, Serbia
Received:

27 December 2011

Accepted:

8 March 2012

Published online:

7 April 2012

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Abstract

Renal tubular acidosis (RTA) is common in adults with primary Sjogren syndrome (pSS) but to date this condition has only been identified in 12 pediatric cases of pSS. Here we present the case of a 13-year-old, otherwise asymptomatic girl in whom the search for the etiology of incidentally found nephrocalcinosis led to diagnosis of distal RTA and nephrogenic diabetes insipidus secondary to SS-associated tubulointerstitial nephritis. Immunosupressive treatment and alkali/electrolyte supplementation resulted in stable renal function over the 6-year follow-up. A review of the literature focuses on two aspects of pSS: (1) the difficulties in diagnosing pSS in childhood and (2) clinical?pathological features, treatment and outcome of renal tubulointerstitial disease in childhood pSS. SS should be considered in older children, particularly females with otherwise unexplained RTA. A careful search for other renal dysfunctions is necessary, and renal biopsy may be of value in assessing the extent of renal damage and the need for immunomodulatory therapy.

Key words

Children - Nephrocalcinosis - Renal tubular acidosis - Sjogren syndrome - Tubulointerstitial nephritis