Mortality in systemic sclerosis

Clin Exp Rheumatol. Sep-Oct 2008;26(5 Suppl 51):S85-93.


Systemic sclerosis is a rare and potentially devastating connective tissue disease. It is highly heterogeneous in terms of clinical presentation, extent and severity of organ involvement, immunologic abnormalities, and clinical course. Although clinical outcomes appear to have improved in recent years, the disease continues to cause substantial excess mortality. In this review, we have systematically collected the published studies addressing the mortality burden in patients with scleroderma in comparison with the general population, as well as studies exploring the most important potential predictors of mortality. Results of these studies are presented and discussed, with emphasis on methodological limitations. Suggestions are made for the design, conduct, and reporting of further research on these themes.

Publication types

  • Review

MeSH terms

  • Age Factors
  • Biomarkers / blood
  • Cause of Death
  • Epidemiologic Research Design
  • Female
  • Humans
  • Male
  • Odds Ratio
  • Scleroderma, Diffuse / mortality*
  • Scleroderma, Limited / mortality*
  • Sex Factors


  • Biomarkers