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Primary Sjögren syndrome in the paediatric age: a multicentre survey

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Abstract

Primary Sjögren syndrome (SS) is very rare in childhood. We collected a series of primary paediatric SS cases from different centres. A data collection form was prepared and sent to rheumatologists who were willing to participate. Data on 40 cases of primary SS with onset before the 16th birthday were collected. Almost all patients (35/40) were females, age at onset varied from 9.3 to 12.4 years (mean 10.7 years). Signs and symptoms at disease onset were mainly recurrent parotid swelling followed by sicca symptoms. Abnormal laboratory tests were found in the majority of cases. Regarding treatment, 22 patients were treated at some time with oral corticosteroids, seven with non-steroidal anti-inflammatory drugs, and five with hydroxychloroquine; two patients needed cyclosporine and one cyclophosphamide. Follow-up varied from 0 to 7.5 years from onset, without major complications in the majority of patients. Conclusion: recurrent parotid swelling is a common feature of primary Sjögren syndrome in childhood and often occurs as a presenting feature. Sicca symptoms may be rarer.

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Abbreviations

ANA :

antinuclear antibodies

ENA :

extractable nuclear antigens

ESR :

erythrocyte sedimentation rate

NSAID :

non-steroidal anti-inflammatory drugs

RF :

rheumatoid factor

SS :

Sjögren syndrome

References

  1. Anaya JM, Ogawa N, Talal N (1995) Sjögren's syndrome in childhood. J Rheumatol 22: 1152–1158

    CAS  PubMed  Google Scholar 

  2. Bartunkova J, Sediva A, Vencovsky J, Tesar V (1999) Primary Sjögren's syndrome in children and adolescents: proposal for diagnostic criteria. Clin Exp Rheumatol 17: 381–386

    CAS  PubMed  Google Scholar 

  3. Berman JL, Kashii S, Trachtman MS, Burde RM (1990) Optic neuropathy and central nervous system disease secondary to Sjögren's syndrome in a child. Ophthalmology 97: 1606–1609

    CAS  PubMed  Google Scholar 

  4. Celada A, Beck D, Chavaz P, Kapanci Y, Cruchaud A (1980) Hypergammaglobulinemic purpura and Sjögren's syndrome in a child. Helv Paediatr Acta 35: 569–576

    CAS  PubMed  Google Scholar 

  5. Chang YC, Huang CC, Chiou YY, Yu CY (1995) Renal tubular acidosis complicated with hypokalemic periodic paralysis. Pediatr Neurol 13: 52–54

    Article  CAS  PubMed  Google Scholar 

  6. Chudwin DS, Daniels TE, Wara DW, Ammann AJ, Barret DJ, Whitcher JP, Cowan MJ (1981) Spectrum of Sjögren's syndrome in children. J Pediatr 98: 213–217

    CAS  PubMed  Google Scholar 

  7. DeGuzman M, Fishman MA, Lewis RA, McCluggage C, Warren RW, Finegold MJ (1998) Chronic neurologic disease with visual, gait, and bladder problems in a male teenager. J Pediatr 132: 742–747

    CAS  PubMed  Google Scholar 

  8. Drosos AA, Tsiakou EK, Tsifetaki N, Politi EN, Siamopoulou-Mavridou A (1997) Subgroups of primary Sjögren's syndrome in male and paediatric Greek patients. Ann Rheum Dis 56: 333–335

    CAS  PubMed  Google Scholar 

  9. Hara T, Nagata M, Mizuno Y, Ura Y, Matsuo M, Ueda K (1992) Recurrent parotid swelling in children: clinical features useful for differential diagnosis of Sjögren's syndrome. Acta Paediatr 81: 547–549

    CAS  PubMed  Google Scholar 

  10. Hearth-Holmes M, Baethge BA, Abreo F, Wolf RE (1993) Autoimmune exocrinopathy presenting as recurrent parotitis of childhood. Arch Otolaryngol Head Neck Surg 119: 347–349

    CAS  PubMed  Google Scholar 

  11. Jay MS, Freeman D, Jamieson D, Wray BB, Durant RH (1986) Sjogren's syndrome in an adolescent. J Adolescent Health Care 7: 53–56

    CAS  Google Scholar 

  12. Kobayashi I, Furuta H, Tame A, Kawamura N, Kojima K, Endoh M, Okano M, Sakiyama Y (1996) Complications of childhood Sjögren syndrome. Eur J Pediatr 155: 890–894

    Article  CAS  PubMed  Google Scholar 

  13. Kraus A, Alarcon-Segovia D (1988) Primary juvenile Sjögren's syndrome. J Rheumatol 15: 803–806

    CAS  PubMed  Google Scholar 

  14. Kumon K, Satake A, Mizumoto M, Kobayashi I, Ishikawa N (2000) A case of sensory neuropathy associated with childhood Sjögren's syndrome. Eur J Pediatr 159: 630–631

    CAS  PubMed  Google Scholar 

  15. Manthorpe R (1996) Sudden onset unilateral renomegaly as an initial manifestation of primary Sjögren's syndrome in a teenage girl. Scand J Rheumatol 25: 186–188

    CAS  PubMed  Google Scholar 

  16. Manthorpe T, Manthorpe R (1992) Congenital complete heart block in children of mothers with primary Sjögren's syndrome. Lancet 340: 1359–1360

    CAS  Google Scholar 

  17. Mizuno Y, Hara T, Hatae K, Hirano T, Ueda K, Nakamura N, Mizoguchi Y (1989) Recurrent parotid gland enlargement as an initial manifestation of Sjögren's syndrome in children. Eur J Pediatr 148: 414–416

    CAS  PubMed  Google Scholar 

  18. Nagahiro S, Mantani A, Yamada K, Ushio Y (1996) Multiple cerebral arterial occlusion in a young patient with Sjögren's syndrome: case report. Neurosurgery 38: 592–595

    CAS  PubMed  Google Scholar 

  19. Ohtsuka T, Saito Y, Hasegawa M, Tatsuno M, Takita S, Arita M, Okuyama K (1995) Central nervous system disease in a child with primary Sjögren's syndrome. J Pediatr 127: 961–963

    CAS  PubMed  Google Scholar 

  20. Ostuni PA, Ianniello A, Sfriso P, Mazzola G, Andretta M, Gambari PF (1996) Juvenile onset of primary Sjögren's syndrome: report of 10 cases. Clin Exp Rheumatol 14: 689–693

    CAS  PubMed  Google Scholar 

  21. Saito T, Fukuda H, Takashi N, Horikawa M, Shindoh M, Anemiya A (1994) Sjögren's syndrome in the adolescent. Oral Pathol 77: 368–372

    CAS  Google Scholar 

  22. Stiller M, Golder W, Döring E, Kliern K (1999) Diagnostic value of scialography with both the conventional and digital subtraction techniques in children with primary and secondary Sjögren's syndrome. Oral Surg Oral Med Oral Pathol 88: 620–627

    CAS  Google Scholar 

  23. Tomiita M, Saito K, Kohno Y, Shimojo N, Fujikawa S, Niimi H (1997) The clinical features of Sjögren's syndrome in Japanese children. Acta Paediatr Japn 39: 268–272

    CAS  Google Scholar 

  24. Vermylen C, Meurant A, Noël H, Claus D, Cornu G (1985) Sjögren's syndrome in a child. Eur J Pediatr 144: 266–269

    CAS  PubMed  Google Scholar 

  25. Yoshida K (1996) Membranous glomerulonephritis with primary Sjögren's syndrome detected by urine screening of school children. Clin Nephrol 45: 422

    CAS  PubMed  Google Scholar 

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Acknowledgements

This study has been partly supported by Grants N. 111300001 from the Czech Ministry of Education and N. 00000064203 from the Czech Ministry of Health.

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Authors and Affiliations

  1. Clinica Pediatrica, Istituti Clinici di Perfezionamento, Via Commenda 9, 20122, Milano, Italy

    Rolando Cimaz & Annachiara Casadei

  2. Du Pont Hospital for Children, Wilmington, USA

    Carlos Rose

  3. Institute of Immunology, Prague, Czech Republic

    Jirina Bartunkova & Anna Sediva

  4. Meyer Hospital, Firenze, Italy

    Fernanda Falcini

  5. G. Gaslini Institute, Genova, Italy

    Paolo Picco

  6. Spedali Civili, Brescia, Italy

    Marco Taglietti

  7. Department of Paediatrics, Padova, Italy

    Francesco Zulian

  8. University Hospital Leiden, Leiden, The Netherlands

    Rebecca Ten Cate

  9. University of the State of Rio de Janeiro, Rio de Janeiro, Brazil

    Flavio R. Sztajnbok

  10. Division of Rheumatology, Department of Internal Medicine, Medical School, University of Ioannina, Greece

    Paraskevi V. Voulgari & Alexandros A. Drosos

Authors
  1. Rolando Cimaz
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  2. Annachiara Casadei
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  3. Carlos Rose
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  4. Jirina Bartunkova
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  5. Anna Sediva
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  7. Paolo Picco
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  9. Francesco Zulian
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  11. Flavio R. Sztajnbok
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  12. Paraskevi V. Voulgari
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  13. Alexandros A. Drosos
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Correspondence to Rolando Cimaz.

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Cimaz, R., Casadei, A., Rose, C. et al. Primary Sjögren syndrome in the paediatric age: a multicentre survey. Eur J Pediatr 162, 661–665 (2003). https://doi.org/10.1007/s00431-003-1277-9

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  • DOI: https://doi.org/10.1007/s00431-003-1277-9

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