A 52-year-old woman with limited systemic sclerosis (lcSSc) develops progressive exertional dyspnoea. Right heart catheterisation shows mean PAP 32 mmHg, PVR 4.2 Wood units, PCWP 10 mmHg. The most likely diagnosis and first-line treatment are:
- A Interstitial lung disease; mycophenolate mofetil
- B Pulmonary venous hypertension; diuretics and ACE inhibitor
- C Pulmonary arterial hypertension; endothelin receptor antagonist (ambrisentan or macitentan) ± PDE5 inhibitor ✓
- D Recurrent pulmonary emboli; anticoagulation
Explanation
The haemodynamic profile (mean PAP ≥25 mmHg, PVR ≥3 Wood units, PCWP ≤15 mmHg) confirms pre-capillary pulmonary arterial hypertension (PAH), a well-recognised complication of lcSSc (CREST syndrome). First-line treatment for Group 1 PAH in SSc includes endothelin receptor antagonists (ambrisentan, macitentan) and/or PDE5 inhibitors (sildenafil, tadalafil); combination upfront therapy is now preferred for WHO functional class II–III based on the AMBITION trial. ILD causes restrictive pattern without pre-capillary PAH profile. Pulmonary venous hypertension would show elevated PCWP >15 mmHg.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.