Systemic sclerosis pulmonary hypertension — SCE Rheumatology MCQ

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ModerateConnective Tissue DiseasesSystemic sclerosis pulmonary hypertensionSCE Rheumatology

A 55-year-old woman with limited cutaneous systemic sclerosis and anticentromere antibodies reports progressive exertional dyspnoea. FVC is 92% predicted, DLCO is 42% predicted and HRCT shows no significant interstitial lung disease. NT-proBNP is raised. What is the most appropriate investigation?

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Correct answer: C — Right heart catheterisation

Explanation lettering: D = shown as A · A = shown as B · B = shown as C · C = shown as D

Right heart catheterisation is best because disproportionately low DLCO and raised NT-proBNP in limited systemic sclerosis raise suspected pulmonary arterial hypertension, confirmed by right heart catheterisation. A is less suitable because bronchoscopy does not diagnose pulmonary arterial hypertension; C is less suitable because temporal artery ultrasound is for suspected GCA; D is less suitable because renal biopsy is not first-line in suspected PAH; E is less suitable because dual-energy CT is a gout investigation and irrelevant. Clinical pearl: echocardiography screens, but right heart catheterisation confirms PAH.

Reference: BSR systemic sclerosis guideline 2024; EULAR systemic sclerosis recommendations 2024