Renal involvement in primary Sjogren syndrome of childhood: case report and literature review.
Mod Rheumatol
; 23(1): 182-9, 2013 Jan.
Article
em En
| MEDLINE
| ID: mdl-22484709
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.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Acidose Tubular Renal
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Síndrome de Sjogren
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Nefrocalcinose
Tipo de estudo:
Diagnostic_studies
/
Prognostic_studies
Limite:
Adolescent
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Female
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Humans
Idioma:
En
Ano de publicação:
2013
Tipo de documento:
Article