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From Localized Scleroderma to Systemic Sclerosis: Coexistence or Possible Evolution.

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Area of Science:

  • Rheumatology
  • Dermatology
  • Autoimmune Diseases

Background:

  • Systemic sclerosis (SSc) and localized scleroderma (LoS) are distinct conditions.
  • These diseases may share overlapping features, necessitating investigation into their relationship.
  • Previous observations suggest potential links, particularly in pediatric cases.

Purpose of the Study:

  • To evaluate the coexistence of SSc and LoS in a cohort of SSc patients.
  • To identify potential risk factors or preceding conditions for developing both diseases.

Main Methods:

  • Retrospective analysis of clinical records from 330 patients diagnosed with SSc.
  • Identification of patients with a documented history of both LoS and SSc.
  • Assessment of the temporal relationship and presence of specific biomarkers.

Main Results:

  • Eight (2.4%) female SSc patients had a history of LoS.
  • Six of these patients developed LoS before SSc, with 4/6 showing autoantibodies prior to SSc diagnosis.
  • The median time between LoS and SSc diagnosis was 18 months.

Conclusions:

  • Systemic sclerosis and localized scleroderma are distinct but can coexist.
  • Adults with LoS may develop SSc, particularly if they have Raynaud's phenomenon or antinuclear antibodies.
  • Early identification of these markers in LoS patients could aid in predicting SSc onset.