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Favourable outcome in 135 children with juvenile systemic sclerosis: results of a multi-national survey

I Foeldvari1, M Zhavania, N Birdi

  • 1University Children's Hospital, Hamburg, Germany.

Rheumatology (Oxford, England)
|June 15, 2000
PubMed

Insights

Juvenile systemic sclerosis (jSSc) shows a favorable outcome in most childhood-onset cases. Survival rates are significantly better in jSSc patients compared to adults with systemic sclerosis.

Area of Science:

  • Pediatric Rheumatology
  • Autoimmune Diseases
  • Systemic Sclerosis

Background:

  • Limited knowledge exists regarding the long-term outcomes of juvenile systemic sclerosis (jSSc).
  • Understanding prognostic factors is crucial for managing this rare pediatric condition.

Purpose of the Study:

  • To enhance understanding of the clinical course and outcomes in juvenile systemic sclerosis.
  • To identify prognostic factors influencing patient outcomes in jSSc.

Main Methods:

  • A questionnaire-based survey was distributed to 324 pediatric rheumatology centers globally.
  • Data from 135 patients diagnosed with jSSc were collected and analyzed.

Main Results:

  • At a mean follow-up of 5 years, 90% of patients were fully active in daily life.
  • The 4-year survival rate was 95%, with major causes of death including heart and renal failure.
  • Disease remained active in 82 patients, while 16 achieved remission.

Conclusions:

  • Childhood-onset systemic sclerosis demonstrates a generally favorable outcome.
  • Survival in jSSc patients is significantly better than in adult-onset systemic sclerosis.
Abstract

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