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Systemic sclerosis-associated pulmonary arterial hypertension — SCE Respiratory MCQ

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HardPulmonary Vascular DiseaseSystemic sclerosis-associated pulmonary arterial hypertensionSCE Respiratory

A 32-year-old woman with systemic sclerosis has exertional dyspnoea. Spirometry shows FVC 92% predicted, TLCO 38% predicted and FVC/TLCO ratio is high. HRCT shows minimal ILD and echocardiography estimates elevated pulmonary pressures. What is the most appropriate investigation?

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Correct answer: B — Right heart catheterisation at a pulmonary hypertension centre

Systemic sclerosis with disproportionate TLCO reduction and echo suspicion requires RHC to confirm and classify pulmonary hypertension. Starting PAH therapy without haemodynamic confirmation risks misclassification and harm. Airway challenge, delayed HRCT and pleural aspiration do not answer the key vascular question.

Reference: https://www.brit-thoracic.org.uk/quality-improvement/guidelines/