A 55-year-old woman with long-standing diffuse cutaneous systemic sclerosis develops exertional dyspnoea. Right heart catheterisation reveals mean pulmonary arterial pressure 35 mmHg and pulmonary capillary wedge pressure 12 mmHg. Which antibody is most characteristically associated with pulmonary arterial hypertension in systemic sclerosis?

• A Anti-Scl-70 (anti-topoisomerase I)
• B Anti-RNA polymerase III
• C Anti-U1-RNP
• D Anti-centromere antibody ✓

Explanation

Pulmonary arterial hypertension (PAH) is the leading cause of death in limited cutaneous systemic sclerosis (lcSSc) and is most strongly associated with anti-centromere antibodies (ACA). Anti-Scl-70 is associated with diffuse cutaneous SSc and pulmonary fibrosis (ILD). Anti-RNA polymerase III correlates with diffuse SSc and renal crisis. Anti-U1-RNP is seen in MCTD with features of SSc.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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