Pulmonary arterial hypertension is a leading cause of death in systemic sclerosis, and symptoms appear late. Annual screening finds it earlier.
Combine symptoms with pulmonary function tests including DLCO, echocardiography and NT-proBNP, or use a structured algorithm such as DETECT. A falling DLCO out of proportion to lung volumes, or a raised tricuspid regurgitant velocity, should prompt referral for right heart catheterisation.
- Screen every patient with systemic sclerosis for PAH yearly.
- Track DLCO and its ratio to FVC over time.
- Use echocardiography and NT-proBNP alongside pulmonary function.
- Refer for right heart catheterisation when screening is positive.
Why it matters
PAH found by screening is diagnosed earlier and at a more treatable stage.
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