A 42-year-old woman with limited systemic sclerosis (CREST syndrome) develops progressive exertional dyspnea. Right heart catheterization shows mean pulmonary arterial pressure 32 mmHg, pulmonary capillary wedge pressure 10 mmHg, pulmonary vascular resistance 4.2 WU. What is the diagnosis and what autoantibody is classically associated?

• A Pulmonary venous hypertension; anti-Scl-70 antibody
• B ILD-associated pulmonary hypertension; anti-RNA polymerase III antibody
• C Pulmonary arterial hypertension; anti-topoisomerase I (Scl-70) antibody
• D Pulmonary arterial hypertension; anti-centromere antibody (ACA) ✓

Explanation

The hemodynamic profile (mPAP ≥25 mmHg, PCWP ≤15 mmHg, PVR >3 WU) defines pulmonary arterial hypertension (group 1 PH). Limited SSc (CREST) is strongly associated with anti-centromere antibody and carries a particularly high risk of PAH. Anti-Scl-70 (topoisomerase I) is associated with diffuse SSc and ILD, which causes group 3 PH (post-capillary hypoxic PH). Anti-RNA pol III is associated with diffuse SSc and renal crisis.

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

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Written and medically reviewed by the StethoPrep medical team.