Rheumatology Advance Access published online on September 13, 2005
Rheumatology, doi:10.1093/rheumatology/kei110
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1 Department of Pediatrics, Divisions of Rheumatology, The Children's Hospital of Philadelphia, PA
* To whom correspondence should be addressed. 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.
Received May 22, 2005
Accepted August 9, 2005
Original Papers
The spectrum of renal tubular acidosis in paediatric Sjögren syndrome
2 Division of Rheumatology, Children's Hospital and Regional Medical Center, Seattle, WA, USA
3 Division of Rheumatology, Department of Internal Medicine, The 2nd Affiliated Hospital, Sun Yat-sen University, Guangzhou 510120, People's Republic of China.
4 University of Pennsylvania School of Medicine, Philadelphia, PA
5 Department of Pediatrics, Divisions of Rheumatology, The Children's Hospital of Philadelphia, PA; University of Pennsylvania School of Medicine, Philadelphia, PA; These two author contributed equally.
6 Department of Pediatrics, Divisions of Nephrology, The Children's Hospital of Philadelphia, PA; University of Pennsylvania School of Medicine, Philadelphia, PA; These two author contributed equally.
F. Pessler, E-mail: pessler{at}email.chop.edu
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