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Mortality in systemic sclerosis: a comparison with the general population

M Abu-Shakra1, P Lee

  • 1University of Toronto Rheumatic Disease Unit, Wellesley Hospital, Canada.

The Journal of Rheumatology
|November 1, 1995
PubMed
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Patients with systemic sclerosis (SSc) face a significantly higher mortality rate compared to the general population. This increased risk is particularly pronounced in individuals with the diffuse form of the disease.

Area of Science:

  • Rheumatology
  • Epidemiology
  • Public Health

Background:

  • Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis and vascular abnormalities.
  • Understanding the long-term survival of SSc patients is crucial for clinical management and resource allocation.

Purpose of the Study:

  • To compare the mortality rate in patients diagnosed with systemic sclerosis (SSc) against the general population.
  • To identify specific SSc subtypes with differential mortality risks.

Main Methods:

  • Prospective cohort study involving 237 SSc patients.
  • Calculation of standardized mortality ratios (SMR) using age- and sex-specific mortality data from Ontario (1976-1990).

Main Results:

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  • The overall SMR for the SSc cohort was 4.69, indicating a nearly fivefold increased mortality risk.
  • Mortality was significantly higher in patients with diffuse SSc (SMR 6.18) compared to limited SSc (SMR 3.80).
  • No significant difference in mortality was observed between male and female SSc patients.

    • Conclusions:

    • Systemic sclerosis is associated with a substantially increased mortality rate compared to the general population.
    • The diffuse subtype of SSc carries a particularly elevated risk of mortality.
    • These findings underscore the need for intensified monitoring and treatment strategies for SSc patients, especially those with diffuse disease.