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Scleroderma pulmonary hypertension — SCE Rheumatology MCQ

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ModerateConnective tissue diseasesScleroderma pulmonary hypertensionSCE Rheumatology

A 59-year-old woman with limited systemic sclerosis has worsening exertional breathlessness. Examination shows lungs are clear and there is a loud pulmonary component of the second heart sound. Investigations show transfer factor is disproportionately low relative to FVC. What is the most appropriate investigation?

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Correct answer: D — Transthoracic echocardiography

Disproportionately low transfer factor and a loud P2 suggest pulmonary arterial hypertension, for which echocardiography is a screening investigation. Creatine kinase or Schirmer testing would not assess pulmonary vascular disease. Right-heart catheterisation confirms pulmonary hypertension after non-invasive screening.

Reference: BSR systemic sclerosis guideline; EULAR systemic sclerosis recommendations