Pulmonary arterial hypertension and systemic sclerosis relation: a single centre experience

Area of Science:

• Cardiology
• Rheumatology
• Pulmonology

Background:

• Systemic sclerosis (SSc) is a complex autoimmune disease.
• Pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) are significant complications in SSc.
• Understanding the prevalence and associations of PAH in SSc is crucial for patient management.

Purpose of the Study:

• To determine the frequency of pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc).
• To investigate PAH occurring alone (SSc-PAH) or with interstitial lung disease (ILD-PH).
• To explore associations between PAH and SSc subtypes.

Main Methods:

• Retrospective review of 141 SSc cases (1990-2011).
• Data collected included clinical findings, skin scores, functional tests (6MWD, DLCO), and imaging (HRCT, echocardiography, right heart catheterisation).
• Comparison between diffuse cutaneous SSc (DcSSc) and limited cutaneous SSc (LcSSc) forms.

Main Results:

• PAH was detected in 24.1% of SSc patients (34/141).
• Seven patients had SSc-PAH, and 27 had ILD-PH; frequencies were similar in DcSSc and LcSSc.
• Higher mean pulmonary arterial pressure (sPAP) was observed in SSc-PAH.

Conclusions:

• PAH is a common complication in SSc, affecting approximately one-fourth of patients.
• Routine advanced cardio-pulmonary investigations are recommended for SSc patients.
• Early detection and management of PAH in SSc can improve patient outcomes.