Pulmonary arterial hypertension is a leading cause of death in systemic sclerosis and is often advanced by the time breathlessness is reported. Annual screening is standard: ask about exertional breathlessness, check pulmonary function including DLCO, measure NT-proBNP, and arrange echocardiography. A falling DLCO out of proportion to lung volumes, or a raised NT-proBNP, warrants referral for right heart catheterisation, which remains the only way to confirm the diagnosis.
- Screen every patient with systemic sclerosis for PAH at least yearly.
- Include pulmonary function with DLCO, NT-proBNP and echocardiography.
- Treat a DLCO falling out of proportion to lung volumes as a warning sign.
- Refer suspected PAH for right heart catheterisation to confirm the diagnosis.
Why it matters
Early detection is what makes early treatment possible.
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