Pulmonary vascular dysfunction in systemic sclerosis

Area of Science:

• Cardiology
• Rheumatology
• Pulmonology

Background:

• Systemic sclerosis (SSc) is associated with a high risk of pulmonary arterial hypertension (PAH).
• Current screening methods for PAH in SSc may not be fully sensitive.
• Early detection of PAH is crucial for timely intervention and improved outcomes.

Purpose of the Study:

• To evaluate the effectiveness of the DETECT algorithm and invasive hemodynamic exercise testing in identifying missed PAH cases in SSc patients undergoing routine screening.
• To assess the prevalence of exercise-induced pulmonary hypertension and group 2 pulmonary hypertension in this cohort.

Main Methods:

• A single-center study of 73 SSc patients without known PAH.
• Echocardiography and the DETECT algorithm were used for initial screening.
• Invasive hemodynamic assessment at rest and during exercise was performed in a subset of patients.

Main Results:

• The DETECT algorithm identified 3 patients meeting 2022 ESC guidelines for PAH at rest.
• 18/19 patients showed abnormal pulmonary arterial pressure during exercise, with 7/9 having exercise-induced pulmonary hypertension.
• One-quarter of patients had group 2 pulmonary hypertension due to left ventricular dysfunction.

Conclusions:

• Routine PAH screening in SSc may miss cases, highlighting the need for enhanced diagnostic approaches.
• The DETECT algorithm and invasive hemodynamic exercise testing can improve PAH detection in SSc.
• Abnormal pulmonary vascular responses during exercise and group 2 PH are common in SSc patients.