A 40-year-old woman with limited cutaneous systemic sclerosis (lcSSc) develops exertional dyspnea. Echo shows estimated RVSP of 52 mmHg and right heart catheterization confirms mPAP 32 mmHg, PCWP 12 mmHg, PVR 4.2 Wood units. The most important initial investigation to guide prognosis in this SSc-PAH patient is:

• A NT-proBNP level ✓
• B 6-minute walk distance
• C Anti-centromere antibody titer
• D HRCT of the thorax

Explanation

NT-proBNP is the most powerful prognostic biomarker in pulmonary arterial hypertension, including SSc-PAH, correlating with right ventricular strain, functional class, and survival. Serial NT-proBNP guides treatment response assessment. The 6-minute walk test provides functional status but is less specific for prognosis in SSc-PAH compared to NT-proBNP. HRCT evaluates parenchymal ILD rather than vascular PAH. Anti-centromere antibody predicts risk of developing PAH in lcSSc but does not guide ongoing prognosis.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

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Written and medically reviewed by the StethoPrep medical team.