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Systemic sclerosis pulmonary arterial hypertension — SCE Rheumatology MCQ

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HardConnective tissue diseasesSystemic sclerosis pulmonary arterial hypertensionSCE Rheumatology

A 48-year-old woman with limited cutaneous systemic sclerosis reports worsening exertional breathlessness. HRCT chest shows minimal fibrosis, echocardiography estimates raised pulmonary artery systolic pressure, NT-proBNP is elevated and spirometry shows preserved FVC with disproportionately reduced DLCO. What is the most appropriate investigation?

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Correct answer: E — Right heart catheterisation

Systemic sclerosis with disproportionate reduction in DLCO, raised NT-proBNP and abnormal echocardiography raises concern for pulmonary arterial hypertension. Right heart catheterisation confirms the diagnosis and guides treatment. HRCT helps assess ILD, but it has not explained the haemodynamic pattern.

Reference: BSR systemic sclerosis guideline 2024; ESC/ERS pulmonary hypertension guideline