Medicine · Rheumatology (SLE, RA, Vasculitis, Crystal Arthropathies, Scleroderma)

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)
Correct answer: 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.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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