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Mortality in systemic sclerosis (scleroderma).

P Lee1, P Langevitz, C A Alderdice

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

The Quarterly Journal of Medicine
|February 1, 1992
PubMed
Summary
This summary is machine-generated.

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Systemic sclerosis survival rates decline over time, with renal, cardiac, and pulmonary issues impacting prognosis. Non-scleroderma causes like cancer also contribute to mortality in patients.

Area of Science:

  • Rheumatology
  • Clinical Medicine
  • Epidemiology

Background:

  • Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis, vascular abnormalities, and immune dysregulation.
  • Prognosis and survival rates in systemic sclerosis patients are influenced by various clinical factors.
  • Understanding long-term outcomes is crucial for patient management and healthcare planning.

Purpose of the Study:

  • To prospectively evaluate the long-term survival rates of patients with systemic sclerosis.
  • To identify prognostic factors associated with reduced survival in systemic sclerosis.
  • To determine the primary causes of mortality in this patient cohort.

Main Methods:

  • Prospective follow-up of 237 patients diagnosed with systemic sclerosis.

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  • Analysis of survival data at 3, 6, and 9 years post-enrollment.
  • Identification of risk factors including organ involvement, age, and sex.

    • Main Results:

    • Overall 3, 6, and 9-year survival rates were 86%, 76%, and 61% respectively.
    • Adverse prognostic factors included renal, cardiac, and pulmonary disease, and older age at enrollment.
    • Pulmonary hypertension was the most common cause of scleroderma-related death; 28% of deaths were unrelated to SSc, including cancer and ischemic heart disease.

    Conclusions:

    • Systemic sclerosis survival is significantly influenced by organ-specific complications and patient age.
    • Pulmonary hypertension is a major determinant of mortality in systemic sclerosis.
    • Non-scleroderma related conditions represent a substantial proportion of deaths, particularly in older patients.