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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Mapping and predicting mortality from systemic sclerosis.

Muriel Elhai1, Christophe Meune2, Marouane Boubaya3

  • 1Rheumatology A department, Paris Descartes University, INSERM U1016, Sorbonne Paris Cité, Cochin Hospital, Paris, France.

Annals of the Rheumatic Diseases
|August 25, 2017
PubMed
Summary
This summary is machine-generated.

Systemic sclerosis (SSc) mortality is primarily due to cardiopulmonary causes. A new risk score accurately predicts 3-year survival, aiding patient management and potentially prolonging life.

Keywords:
cardiovascular diseaseepidemiologypulmonary fibrosissystemic sclerosis

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Area of Science:

  • Rheumatology
  • Cardiology
  • Pulmonology

Background:

  • Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis of the skin and internal organs.
  • Understanding the causes of death and identifying risk factors are crucial for improving patient outcomes in SSc.

Purpose of the Study:

  • To determine the primary causes of mortality in patients with systemic sclerosis.
  • To identify risk factors associated with all-cause mortality in SSc.
  • To develop a predictive risk score for 3-year mortality in SSc patients.

Main Methods:

  • Analysis of 2719 French death certificates for SSc patients (2000-2011).
  • Examination of causes of death and development of a risk score using the international European Scleroderma Trials and Research (EUSTAR) database.
  • Prognostic factors were tested using Cox regression models, with internal validation via bootstrapping.

Main Results:

  • Cardiac (31%) and respiratory (18%) causes were the leading causes of death in French SSc patients.
  • In the EUSTAR cohort, cardiac (27%) and respiratory (17%) causes predominated mortality.
  • A validated risk score accurately predicted 3-year mortality (AUC=0.82), with significant survival differences between risk quartiles (53% vs. 98%).

Conclusions:

  • The cardiopulmonary system is the major contributor to SSc mortality.
  • A robust risk score has been developed to stratify SSc patients and estimate 3-year survival.
  • These findings can guide clinical management and monitoring to improve survival in SSc patients, especially with emerging therapies.