Multimodal Screening for Pulmonary Arterial Hypertension in Systemic Scleroderma: Current Methods and Future...

Area of Science:

• Rheumatology
• Cardiology
• Pulmonology

Background:

• Systemic sclerosis (SSc) is an immuno-inflammatory disease causing fibrosis affecting organs.
• Pulmonary arterial hypertension (PAH) is a severe complication of SSc, increasing mortality.
• Right heart catheterization (RHC) is the gold standard for PAH diagnosis but is invasive.

Purpose of the Study:

• To review non-invasive screening methods for SSc-PAH.
• To highlight the importance of early PAH detection in SSc patients.
• To discuss the role of echocardiography, biomarkers, and pulmonary function tests.

Main Methods:

• Review of current literature on SSc-PAH screening.
• Analysis of echocardiography's utility in assessing right heart structure and function.
• Evaluation of serum biomarkers (NT-proBNP, uric acid, novel markers) and pulmonary function tests.

Main Results:

• Echocardiography is a key non-invasive tool for SSc-PAH screening.
• Serum biomarkers, including novel ones, aid in risk identification and prognosis.
• Pulmonary function tests assist in selecting patients for RHC.

Conclusions:

• Non-invasive screening is crucial for SSc-PAH due to RHC invasiveness.
• A multi-modal approach using echocardiography, biomarkers, and PFTs improves SSc-PAH detection.
• Early identification facilitates timely intervention and improves outcomes for SSc patients with PAH.