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End-stage renal failure in adolescence with Sjögren’s syndrome autoantibodies SSA and SSB

Pediatric Nephrology volume 22, pages 1793–1797 (2007)Cite this article

Abstract

We describe two adolescents who presented with end-stage renal failure and clinical features suggestive of Sjögren’s syndrome (SS). They both demonstrated severe, chronic, tubulointerstitial inflammation on renal biopsy, high-titre antinuclear antibodies, high immunoglobulin A and G concentrations, positive anti-SSA and anti-SSB antibodies, and negative anti-double-stranded DNA antibodies. One had subjective and objective evidence of the sicca complex (dry eyes and/or dry mouth) and fulfilled the commonly accepted SS consensus criteria. The other showed no evidence of the sicca complex but fulfilled modified criteria for juvenile SS. SS may be underrecognised as a cause of end-stage renal failure in childhood.

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Affiliations

  1. Department of Nephrology, Birmingham Children’s Hospital, Birmingham, B4 6NH, UK

    Sally Johnson, Sally-Anne Hulton & C. Mark Taylor

  2. Department of Histopathology, Birmingham Children’s Hospital, Birmingham, B4 6NH, UK

    Marie-Anne Brundler

  3. Department of Dermatology, Birmingham Children’s Hospital, Birmingham, B4 6NH, UK

    Celia Moss

  4. Department of Immunology, Birmingham Heartlands Hospital, Birmingham, B9 5SS, UK

    Aarnoud Huissoon

  5. Department of Renal Immunobiology, University of Birmingham, Birmingham, B15 2TT, UK

    Sally Johnson

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  1. Sally Johnson
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  2. Sally-Anne Hulton
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  3. Marie-Anne Brundler
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  4. Celia Moss
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  5. Aarnoud Huissoon
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  6. C. Mark Taylor
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Correspondence to Sally Johnson.

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Johnson, S., Hulton, SA., Brundler, MA. et al. End-stage renal failure in adolescence with Sjögren’s syndrome autoantibodies SSA and SSB. Pediatr Nephrol 22, 1793–1797 (2007). https://doi.org/10.1007/s00467-007-0526-y

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  • DOI: https://doi.org/10.1007/s00467-007-0526-y

Keywords

  • Sjögren’s syndrome
  • Tubulointerstitial nephritis
  • Extraglandular manifestations
  • C1 esterase inhibitor deficiency
  • Renal failure