Screening for pulmonary arterial hypertension in systemic sclerosis

Area of Science:

• Cardiology
• Rheumatology
• Pulmonology

Background:

• Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis (SSc), affecting about 10% of patients.
• PAH diagnosis in SSc often occurs late, with patients presenting severe symptoms and poor survival rates.
• Early detection of PAH in SSc is vital for potentially improving clinical outcomes.

Purpose of the Study:

• To review current evidence and strategies for the early screening and diagnosis of PAH in SSc patients.
• To highlight the limitations of existing screening methods and suggest pathways for further investigation.

Main Methods:

• Review of current literature on PAH screening in SSc.
• Discussion of diagnostic tools including echocardiography, DETECT algorithm, pulmonary function tests, and N-terminal-pro-brain natriuretic peptide levels.
• Consideration of right heart catheterisation as a gold standard for diagnosis in specific patient groups.

Main Results:

• Annual screening may involve echocardiography, but visualization or tricuspid regurgitation can limit accuracy.
• Alternative screening methods include the DETECT algorithm or a combination of pulmonary function tests and N-terminal-pro-brain natriuretic peptide.
• Right heart catheterisation is recommended for symptomatic patients or those screening positive on other algorithms.

Conclusions:

• Systematic screening of asymptomatic SSc patients holds potential for early PAH identification.
• While echocardiography is common, other algorithms and biomarkers show promise for earlier detection.
• Further research, including systematic catheterisation studies, is needed to validate predictive values for PAH screening in SSc.