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Systemic sclerosis-associated pulmonary arterial hypertension — SCE Rheumatology MCQ

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ModeratePulmonary MedicineSystemic sclerosis-associated pulmonary arterial hypertensionSCE Rheumatology

A 63-year-old woman with limited cutaneous systemic sclerosis reports 8 months of progressive exertional breathlessness. She is in WHO functional class II. Pulmonary function testing shows FVC 91% predicted and TLCO 46% predicted. High-resolution CT shows no clinically important interstitial lung disease. Ventilation–perfusion scintigraphy excludes chronic thromboembolic disease, and left ventricular systolic and diastolic function are normal. Right heart catheterisation demonstrates a mean pulmonary arterial pressure of 27 mmHg, pulmonary arterial wedge pressure of 10 mmHg and pulmonary vascular resistance of 4.2 Wood units. She has no syncope, right-heart failure, hypotension, hepatic impairment or renal impairment. Which is the most appropriate initial disease-targeted treatment strategy?

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Correct answer: D — Start combined phosphodiesterase-5 inhibitor and endothelin receptor antagonist therapy

Explanation lettering: E = shown as B · B = shown as C · C = shown as E

The catheterisation findings establish precapillary pulmonary hypertension: mean pulmonary arterial pressure is above 20 mmHg, wedge pressure is no greater than 15 mmHg and pulmonary vascular resistance is above 2 Wood units. In systemic sclerosis, exclusion of significant interstitial lung disease, left-heart disease and chronic thromboembolic disease supports group 1 systemic sclerosis-associated pulmonary arterial hypertension. EULAR recommends initial combination treatment with a phosphodiesterase-5 inhibitor and an endothelin receptor antagonist for systemic sclerosis-associated pulmonary arterial hypertension. This patient is stable and in WHO functional class II, making oral dual therapy the appropriate initial strategy. A and B are plausible because either drug class has historically been used alone, but current systemic sclerosis-specific guidance favours initial combination therapy rather than monotherapy. C is inappropriate because intravenous epoprostenol is principally considered for advanced disease, particularly functional class III–IV or high-risk presentations; this patient has no right-heart failure or haemodynamic instability. E would be appropriate for inflammatory or fibrotic systemic sclerosis-associated interstitial lung disease, but her imaging and preserved FVC do not indicate clinically important ILD, and immunosuppression is not disease-targeted treatment for isolated systemic sclerosis-associated pulmonary arterial hypertension.

Reference: Updated: Recommendations for systemic sclerosis (2023 update; official summary available 2026) — https://www.eular.org/document/download/1021/cb11a479-9d7a-4b7e-8020-b8cc1dc69bb8/932