skip to main content

SSc-PAH Classification and Treatment — EECC MCQ

Instant feedback + full explanation. One question, done properly.

ModeratePulmonary Vascular DiseaseSSc-PAH Classification and TreatmentEECC

A 55-year-old woman with scleroderma (limited cutaneous SSc) and anti-centromere antibodies develops progressive dyspnoea. RHC confirms PAH (mPAP 35 mmHg, PAWP 10 mmHg, PVR 6 WU). Which PAH group is SSc-PAH classified under and what is the initial treatment approach?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: C — Group 1 PAH (connective tissue disease-associated PAH); initial dual oral combination therapy (ERA + PDE5i) is recommended for intermediate-risk patients, with more aggressive triple therapy for high-risk patients

SSc-PAH is classified as Group 1 PAH (alongside idiopathic PAH, hereditary PAH, drug-induced, and other CTD-PAH). SSc-PAH has a worse prognosis than idiopathic PAH. The 2022 ESC/ERS PH Guidelines recommend: (1) regular PAH screening in SSc patients (annual echocardiography, DLCO — DETECT algorithm); (2) initial combination therapy based on risk stratification: low-intermediate risk → dual oral therapy (ERA such as ambrisentan/macitentan + PDE5 inhibitor such as tadalafil/sildenafil); high risk → initial triple therapy including IV/SC prostacyclin; (3) response assessment at 3-6 months with treatment escalation if not meeting low-risk targets. Early detection (screening) and prompt initiation of combination therapy improve outcomes in SSc-PAH.

Reference: ESC/ERS (2022): PH Guidelines; DETECT Algorithm