A Clinical Decision Tool for Risk Stratifying Patients with Systemic Sclerosis-Related Pulmonary Hypertension

Area of Science:

• Cardiology
• Pulmonology
• Rheumatology

Background:

• Systemic sclerosis (SSc) is associated with a high risk of pulmonary hypertension (PH).
• Early detection of PH in SSc is crucial for timely intervention and improved outcomes.
• Current diagnostic methods can be invasive and may delay treatment.

Purpose of the Study:

• To develop and validate a scoring system for identifying SSc patients at risk of PH.
• To assess the system's ability to predict all-cause mortality in SSc patients with PH.
• To create a simplified clinical decision tool for earlier PH detection in SSc.

Main Methods:

• A scoring system was developed using 7 variables from pulmonary function testing, echocardiography, and chest CT imaging.
• The score was applied to a retrospective cohort of 117 SSc patients.
• Diagnostic performance (sensitivity, specificity, accuracy) for PH was evaluated using right heart catheterization as the gold standard.

Main Results:

• Pulmonary hypertension (PH) was diagnosed in 60 (51.3%) of the SSc patients.
• The scoring system (threshold ≥ 0) demonstrated a sensitivity of 0.87, specificity of 0.74, and accuracy of 0.80 for PH detection.
• Each one-point increase in the score was associated with a 1.19-fold increased adjusted hazard ratio for all-cause mortality.

Conclusions:

• The developed scoring system is a promising tool for identifying SSc patients at risk for PH.
• The score also predicts all-cause mortality, offering prognostic value.
• Further validation in external cohorts is needed to establish this simplified clinical decision tool for routine use in early PH detection in SSc.