Scleroderma in children

Best Pract Res Clin Rheumatol. 2017 Aug;31(4):576-595. doi: 10.1016/j.berh.2018.02.004. Epub 2018 Mar 27.


Juvenile scleroderma with its two varieties, juvenile localized scleroderma and systemic sclerosis (SSc), represents the third most frequent rheumatic disease in childhood. In juvenile SSc, new developments have been recently reported in the fields of classification and monitoring. The introduction of new classification criteria for adult SSc has stimulated new ideas on how to improve the performance of the provisional 2007 PRES/ACR/EULAR pediatric criteria. The introduction of a multidimensional severity score, named "J4S," which includes parameters on growth, skin, and internal organ involvement, has improved the approach to the patients in the daily practice to guide decision-making. In localized scleroderma, the wider application of clinical and instrumental scoring systems has greatly improved both assessment and monitoring. Finally, a multicenter consensus statement and long-term follow-up studies have confirmed the important role of methotrexate for the treatment.

Keywords: Assessment; Classification; Juvenile localized scleroderma; Juvenile systemic sclerosis; Monitoring; Outcome; Outcome measures; Severity score; Treatment.

Publication types

  • Review

MeSH terms

  • Child
  • Decision Making
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Methotrexate / therapeutic use
  • Scleroderma, Localized / complications*
  • Scleroderma, Localized / diagnosis
  • Scleroderma, Localized / drug therapy
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / diagnosis
  • Scleroderma, Systemic / drug therapy


  • Immunosuppressive Agents
  • Methotrexate

Supplementary concepts

  • Juvenile systemic scleroderma