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SSc-PAH Initial Treatment — SCE Rheumatology MCQ

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HardSystemic SclerosisSSc-PAH Initial TreatmentSCE Rheumatology

Right-heart catheterization confirms systemic-sclerosis-associated pulmonary arterial hypertension without high-risk haemodynamic instability. What is the usual initial disease-targeted approach?

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Correct answer: C — Use endothelin-receptor antagonist and PDE5 inhibitor combination

The best answer is “Use endothelin-receptor antagonist and PDE5 inhibitor combination”. Most patients with SSc-associated PAH who are not in a special low-risk or contraindicated group are considered for combination disease-targeted therapy after specialist risk assessment. Oxygen and diuretics are supportive when indicated, monotherapy is not the default for every patient, and glucocorticoids do not treat PAH.

Reference: BSR systemic sclerosis guideline: https://pmc.ncbi.nlm.nih.gov/articles/PMC11534099/