Survival rate, causes of death, and risk factors in systemic sclerosis: a large cohort study

Area of Science:

• Rheumatology
• Internal Medicine
• Clinical Research

Background:

• Systemic sclerosis (SSc) is a complex autoimmune disease with significant morbidity and mortality.
• Understanding the clinical patterns, survival rates, and risk factors in diverse populations is crucial for improving patient outcomes.

Purpose of the Study:

• To investigate the clinical characteristics, survival rates, causes of death, and prognostic factors in a large cohort of Chinese Han patients with SSc.
• To identify independent risk factors associated with mortality in this specific patient group.

Main Methods:

• Retrospective analysis of 201 inpatients diagnosed with SSc between 2002 and 2014.
• Classification into diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc), and SSc-overlap syndrome.
• Statistical analysis including Chi-squared tests, Kaplan-Meier curves, log-rank tests, and Cox proportional hazards modeling.

Main Results:

• Diffuse cutaneous SSc (dcSSc) was the most prevalent subtype (50.2%).
• Interstitial lung disease (ILD) was the most frequent organ involvement (74%), and the leading cause of death, often in combination with infection (50%).
• Overall 5- and 10-year survival rates were 98% and 95%, respectively. Pericardial effusion and digital ischemia were identified as independent predictors of mortality.

Conclusions:

• The mortality rate in SSc patients is slightly elevated compared to the general population.
• ILD is the most common systemic manifestation and the primary driver of mortality in SSc.
• Pericardial effusion and digital ischemia represent significant independent prognostic factors for death in SSc patients.