Which autoantibody in systemic sclerosis (scleroderma) is MOST strongly associated with pulmonary arterial hypertension (PAH) without significant ILD?
- A Anti-Scl-70 (anti-topoisomerase I)
- B Anti-RNA polymerase III
- C Anti-fibrillarin (anti-U3 RNP)
- D Anti-centromere antibody (ACA) ✓
Explanation
Anti-centromere antibody is classically associated with limited cutaneous systemic sclerosis (lcSSc/CREST syndrome), which carries a higher risk of isolated PAH without severe ILD. Anti-Scl-70 is linked to diffuse cutaneous SSc with extensive ILD but lower PAH risk. Anti-RNA polymerase III is associated with diffuse SSc and renal crisis. Anti-fibrillarin is associated with diffuse SSc and pulmonary hypertension in some populations but is not the classic PAH marker.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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