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Scleroderma--demographics and survival

A J Silman1

  • 1ARC Epidemiology Research Unit, University of Manchester, UK.

The Journal of Rheumatology. Supplement
|May 1, 1997
PubMed
Summary
This summary is machine-generated.

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Scleroderma is a rare autoimmune disease affecting connective tissues. Incidence is low, with higher rates in women, and significantly reduced survival, alongside increased cancer mortality.

Area of Science:

  • Rheumatology
  • Epidemiology

Background:

  • Scleroderma is a rare connective tissue disease with limited epidemiological data.
  • Published prevalence estimates vary, often based on hospital-ascertained cases.

Purpose of the Study:

  • To summarize current understanding of scleroderma occurrence and outcomes.
  • To highlight variations in reported prevalence and incidence rates.

Main Methods:

  • Review of published literature on scleroderma prevalence and incidence.
  • Analysis of demographic factors and survival data.

Main Results:

  • Prevalence rates range from 30-120 per million population.
  • Annual incidence is significantly lower, at 2-20 per million per year.

Related Experiment Videos

  • Higher incidence observed in females, peaking in the fifth and sixth decades; 10-year survival is approximately 50%.

    • Conclusions:

    • Scleroderma occurrence data is variable, influenced by study methodology.
    • The disease is associated with reduced survival and increased cancer mortality, particularly lung cancer.