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SSc-PAH — SCE Rheumatology MCQ

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HardSystemic SclerosisSSc-PAHSCE Rheumatology

A 45-year-old woman with limited cutaneous systemic sclerosis develops exertional dyspnoea. Right-heart catheterisation shows a mean pulmonary arterial pressure of 31 mmHg, pulmonary arterial wedge pressure of 10 mmHg and pulmonary vascular resistance of 4.8 Wood units. Significant interstitial lung disease and left-heart disease have been excluded. She is in WHO functional class II, without syncope or right-heart failure. According to the EULAR 2023 systemic sclerosis treatment update, which is the recommended initial disease-targeted treatment approach?

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Correct answer: E — Initial oral dual therapy with an endothelin receptor antagonist and a PDE5 inhibitor

The correct answer is E. The catheter findings establish precapillary pulmonary hypertension, and exclusion of significant lung and left-heart disease supports SSc-associated PAH. The EULAR 2023 update recommends an endothelin receptor antagonist plus a PDE5 inhibitor as first-line treatment at diagnosis. This was supported by SSc-PAH analyses of AMBITION, in which initial ambrisentan–tadalafil combination therapy reduced clinical-failure events compared with either component alone. PDE5-inhibitor or endothelin-receptor-antagonist monotherapy is therefore not the preferred initial strategy. Intravenous epoprostenol is important in advanced SSc-PAH, particularly functional class III–IV disease, but is not appropriate as sole routine first-line therapy in this stable functional class II presentation. Calcium-channel blockers are not an empirical disease-targeted treatment for SSc-PAH.

Reference: Del Galdo F, et al. EULAR recommendations for the treatment of systemic sclerosis: 2023 update. Annals of the Rheumatic Diseases. 2025;84(1):29–40. Pulmonary arterial hypertension section. https://ard.bmj.com/content/early/2024/10/17/ard-2024-226430