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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research
Anthony J Tyndall1, Bettina Bannert, Madelon Vonk
1Department of Rheumatology, University of Basel, Basel, Switzerland.
Systemic sclerosis (SSc) mortality is primarily driven by disease-related complications like pulmonary fibrosis and pulmonary arterial hypertension (PAH). Identifying risk factors such as proteinuria and cardiac issues is crucial for managing SSc patient survival.
Area of Science:
- Rheumatology
- Pulmonology
- Cardiology
- Epidemiology
Background:
- Systemic sclerosis (SSc) is a complex autoimmune disease with significant mortality.
- Understanding the causes and predictors of death in SSc is essential for improving patient outcomes.
Purpose of the Study:
- To investigate the primary causes of mortality in a large cohort of patients with systemic sclerosis.
- To identify independent predictors of mortality in systemic sclerosis.
Main Methods:
- Analysis of 5860 patients from the EULAR Scleroderma Trials and Research (EUSTAR) cohort.
- Utilized Kaplan-Meier and Cox proportional hazards models to assess survival and identify mortality predictors.
- Collected data on causes of death and comorbidities through structured questionnaires.
Main Results:
- Pulmonary fibrosis, pulmonary arterial hypertension (PAH), and cardiac causes were the leading SSc-related causes of death.
- Infections and malignancies were major non-SSc causes of mortality.
- Independent mortality predictors included proteinuria, PAH, pulmonary restriction, dyspnea severity, reduced diffusing capacity, age at Raynaud's onset, and modified Rodnan skin score.
Conclusions:
- Disease-related complications, particularly pulmonary fibrosis, PAH, and cardiac issues, are the predominant causes of death in SSc.
- Several clinical factors independently predict mortality in SSc patients.
- These findings highlight the importance of monitoring specific organ systems and risk factors in SSc management.
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