Systemic Sclerosis — SCE Rheumatology MCQ

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ModerateSystemic SclerosisSystemic SclerosisSCE Rheumatology

A 50-year-old woman with limited cutaneous systemic sclerosis and positive anti-centromere antibodies presents with progressive exertional dyspnoea. Echocardiography estimates a pulmonary artery systolic pressure of 55 mmHg. HRCT shows no significant interstitial lung disease. Which investigation is required to confirm pulmonary arterial hypertension before PAH-specific treatment is considered?

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Correct answer: A — Right heart catheterisation

The correct answer is right heart catheterisation. This patient has a high-risk phenotype for systemic-sclerosis-associated PAH, with limited cutaneous disease, anti-centromere antibodies, progressive dyspnoea, raised echocardiographic pulmonary pressure and no substantial ILD. Echocardiography estimates the probability of pulmonary hypertension but cannot establish the haemodynamic diagnosis or distinguish pre-capillary PAH from left-heart disease. Right heart catheterisation is therefore required before PAH-specific therapy, measuring mean pulmonary arterial pressure, pulmonary artery wedge pressure and pulmonary vascular resistance. A ventilation-perfusion scan is important during the wider pulmonary-hypertension assessment to exclude chronic thromboembolic disease, but it does not confirm PAH. CT pulmonary angiography and cardiopulmonary exercise testing may contribute to differential diagnosis, while delayed repeat echocardiography is inappropriate in a symptomatic patient with a high-probability screening result.

Reference: British Society for Rheumatology. Guideline for the management of systemic sclerosis in people of all ages, 2024 (cardiopulmonary complications/PAH). https://www.rheumatology.org.uk/guidelines/clinicalguidelines/adultsratnfguideline/view