Skip to main content
SpringerLink
Log in

Current developments in pediatric systemic sclerosis

  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Juvenile systemic sclerosis is an orphan disease with an incidence of around 0.05 per 100,000 children. The mean age of onset is approximately 8 years, and 90% of pediatric patients have a diffuse subset. The organ involvement of pediatric patients differs from adult patients. Survival rates are significantly better in pediatric patients than in adult patients. Most patients who die in the first 5 years of the disease have a diffuse subset. Interestingly, the disease subset shifts when pediatric patients are reviewed in adult cohorts of systemic sclerosis patients, in which only around 40% of patients have diffuse subset. The pediatric-onset patients still have a low prevalence of anticentromere antibodies. There is a larger group of patients with overlap features in juvenile systemic sclerosis than in adult cohorts. There are also noticeable differences in organ involvement.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References and Recommended Reading

  1. Zulian F, Woo P, Athreya BH, et al.: The Pediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for juvenile systemic sclerosis. Arthritis Rheum 2007, 57:203–212.

    Article  PubMed  Google Scholar 

  2. Abraham D, Distler O: How does endothelial cell injury start? The role of endothelin in systemic sclerosis. Arthritis Res Ther 2007, 9(Suppl 2):S2.

    Article  PubMed  Google Scholar 

  3. Gabrielli A, Svegliati S, Moroncini G, Avvedimento EV: Pathogenic autoantibodies in systemic sclerosis. Curr Opin Immunol 2007, 19:640–645.

    Article  PubMed  CAS  Google Scholar 

  4. Varga J, Abraham D: Systemic sclerosis: a prototypic multisystem fibrotic disorder. J Clin Invest 2007, 117:557–567.

    Article  PubMed  CAS  Google Scholar 

  5. Gu YS, Kong J, Cheema GS, et al.: The immunobiology of systemic sclerosis. Semin Arthritis Rheum 2008, 38:132–160.

    Article  PubMed  CAS  Google Scholar 

  6. Boin F, Rosen A: Autoimmunity in systemic sclerosis: current concepts. Curr Rheumatol Rep 2007, 9:165–172.

    Article  PubMed  CAS  Google Scholar 

  7. Mayes MD, Trojanowska M: Genetic factors in systemic sclerosis. Arthritis Res Ther 2007, 9(Suppl 2):S2–S5.

    Google Scholar 

  8. Kowal-Bielecka O: EULAR/EUSTAR recommendations for the treatment of systemic sclerosis arthritis and rheumatism. 2007, 56(Suppl):S64–A30.

    Google Scholar 

  9. Masi AT, Rodnan GP, Medsger TA Jr, et al.: Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980, 23:581–590.

    Article  Google Scholar 

  10. LeRoy EC, Medsger TA: Criteria for the classification of early systemic sclerosis. J Rheumatol 2001, 28:1573–1576.

    PubMed  CAS  Google Scholar 

  11. Pelkonen PM, Jalanko HJ, Lantto RK, et al.: Incidence of systemic connective tissue disease in children: a nationwide prospective study in Finland. J Rheumatol 1994, 21:2143–2146.

    PubMed  CAS  Google Scholar 

  12. Arias-Nunez MC, Llorca J, Vazquez-Rodriguez TR, et al.: Systemic sclerosis in northwestern Spain: a 19-year epidemiologic study. Medicine (Baltimore) 2008, 87:272–280.

    Article  Google Scholar 

  13. Martini G, Foeldvari I, Russo R, et al.: Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database. Arthritis Rheum 2006, 54:3971–3978.

    Article  PubMed  CAS  Google Scholar 

  14. Herrick ML, Moore T, Hollis S, Jayson MIV: The influence of age on nailfold capillary dimension in childhood. J Rheumatol 2000, 27:797–800.

    PubMed  CAS  Google Scholar 

  15. Dolezalova P, Young SP, Bacon PA, Southwood TR: Nailfold capillary microscopy in healthy children and in childhood rheumatic diseases: a prospective single blind observational study. Ann Rheum Dis 2003, 62:444–449.

    Article  PubMed  CAS  Google Scholar 

  16. Foeldvari I, Wierk A: Healthy children have a significantly increased skin score assessed with the modified Rodnan skin score. Rheumatology 2006, 45:76–78.

    Article  PubMed  CAS  Google Scholar 

  17. Scalapino K, Arkachaisri T, Lucas M, et al.: Childhood onset systemic sclerosis: classification, clinical and serologic features, and survival in comparison with adult onset disease. J Rheumatol 2006, 33:1004–1013.

    PubMed  Google Scholar 

  18. Vesely R, Vargova V, Ravelli A, et al.: Serum level of KL-6 as a marker of interstitial lung disease in patients with juvenile systemic scleroderma. J Rheumatol 2004, 31:795–800.

    PubMed  Google Scholar 

  19. Lammers AE, Hislop AA, Haworth SG: Prognostic value of B-type natriuretic peptide in children with pulmonary hypertension. Int J Cardiol 2008 Jul 1 (Epub ahead of print).

  20. Foeldvari I, Zhavania M, Birdi N, et al.: Favourable outcome in 135 children with juvenile systemic sclerosis: results of a multi-national survey. Rheumatology (Oxford) 2000, 39:556–559.

    Article  CAS  Google Scholar 

  21. Aoyama K, Nagai Y, Endo Y, Ishikawa O: Juvenile systemic sclerosis: report of three cases and review of Japanese published work. J Dermatol 2007, 34:658–661.

    Article  PubMed  Google Scholar 

  22. Russo R, Katsicas MM: Clinical characteristics of children with juvenile systemic sclerosis: follow up of 23 patients in a single tertiary center. Pediatr Rheum 2007, 5:6.

    Article  Google Scholar 

  23. Misra R, Singh G, Aggarwal P, Aggarwal A: Juvenile onset systemic sclerosis: a single center experience of 23 cases from Asia. Clin Rheumatol 2007, 26:1259–1262.

    Article  PubMed  Google Scholar 

  24. LeRoy EC, Krieg T, Black C, et al.: Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988, 15:202–205.

    PubMed  CAS  Google Scholar 

  25. Martini G, Vittadello F, Kasapcopur O, et al.: Factors affecting survival in juvenile systemic sclerosis. Rheumatology (Oxford) 2009, 48:119–122.

    Article  CAS  Google Scholar 

  26. Czirjak L, Kumanovics G, Varju C, et al.: Survival and causes of death in 366 Hungarian patients with systemic sclerosis. Ann Rheum Dis 2008, 67:59–63.

    Article  PubMed  CAS  Google Scholar 

  27. Foeldvari I, Tyndall A: Juvenile and young adult onset systemic sclerosis share the same outcome and organ involvement: data from the EUSTAR database on an adult cohort of systemic sclerosis patients. Arthritis Rheum 2007, 56(Suppl S53):A1.

    Google Scholar 

  28. Foeldvari I, Nihtyanova S, Wierk A, Denton CP: Characteristics of juvenile onset systemic sclerosis patients in an adult single centre cohort. Arthritis Rheum 2008, 58(Suppl S379):A573.

    Google Scholar 

  29. Walker UA, Tyndall A, Czirjak L, et al.: Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database. Ann Rheum Dis 2007, 66:754–763.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Hamburger Zentrum für Kinder- und Jugendrheumatologie, Kompetenz-Zentrum für Sklerodermie und autoimmune Uveitis im Kindesalter, Am Klinikum Eilbek, Dehnhaide 120, D-22081, Hamburg, Germany

    Ivan Foeldvari

Authors
  1. Ivan Foeldvari
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ivan Foeldvari.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Foeldvari, I. Current developments in pediatric systemic sclerosis. Curr Rheumatol Rep 11, 97–102 (2009). https://doi.org/10.1007/s11926-009-0014-4

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11926-009-0014-4

Keywords

Search

Navigation