Document Detail

The spectrum of renal tubular acidosis in paediatric Sjögren syndrome.
MedLine Citation:
PMID:  16159947     Owner:  NLM     Status:  MEDLINE    
OBJECTIVES: Renal tubular acidosis (RTA) is a well-recognized extraglandular complication of adult Sjögren syndrome (SS) but has been reported only rarely in paediatric SS. We wished to describe the natural history of RTA in paediatric SS. METHODS: We performed a chart and literature review. Inclusion criteria were primary or secondary SS with onset before 18 yr of age, complicated by RTA before 18 yr of age. RESULTS: Twelve cases were identified: two from chart review and 10 from the literature. RTA was mostly associated with primary SS. RTA was detected at the onset of SS or up to 9 yr later. The clinical spectrum ranged from nearly silent to life-threatening, with plasma pH and serum potassium as low as 7.0 and 1.2 mEq/l, respectively. Hypokalaemia was present in 92%. Half the patients presented with profound weakness or paralysis, most likely from hypokalaemia. Proximal, distal and mixed RTA were detected, reflecting a diffuse 'tubulopathy' from interstitial nephritis, which was the predominant histopathological finding. Diabetes insipidus was the most frequent renal comorbidity. The RTA stabilized in 82% of the cases and resolved in one case. Only one patient had long-term unstable RTA. CONCLUSIONS: RTA is an under-recognized complication of paediatric SS. It can be life-threatening in the acute phase but generally has a good long-term renal outcome. SS should be considered in the older child with otherwise unexplained RTA. Likewise, RTA should be excluded in children and adolescents with SS who develop weakness, fatigue or growth failure. Early recognition would reduce long-term complications such as growth failure.
F Pessler; H Emery; L Dai; Y-M Wu; B Monash; R Q Cron; M Pradhan
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review     Date:  2005-09-13
Journal Detail:
Title:  Rheumatology (Oxford, England)     Volume:  45     ISSN:  1462-0324     ISO Abbreviation:  Rheumatology (Oxford)     Publication Date:  2006 Jan 
Date Detail:
Created Date:  2005-12-19     Completed Date:  2006-02-09     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  100883501     Medline TA:  Rheumatology (Oxford)     Country:  England    
Other Details:
Languages:  eng     Pagination:  85-91     Citation Subset:  AIM; IM    
Department of Pediatrics, Division of Rheumatology, The Children's Hospital of Philadelphia, PA 19104, USA.
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MeSH Terms
Acidosis, Renal Tubular / diagnosis,  etiology*
Child, Preschool
Early Diagnosis
Infant, Newborn
Sjogren's Syndrome / complications*
Grant Support

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