Anti-centromere antibodies (ACA) in systemic sclerosis are MOST specifically associated with which subset and its visceral complication?
- A Limited cutaneous SSc (CREST) with pulmonary arterial hypertension ✓
- B Diffuse cutaneous SSc with interstitial lung disease
- C Overlap syndrome with features of myositis
- D Diffuse SSc with renal crisis
Correct answer: A. Limited cutaneous SSc (CREST) with pulmonary arterial hypertension
Explanation
Anti-centromere antibodies are the hallmark autoantibodies of limited cutaneous systemic sclerosis (lcSSc/CREST syndrome: Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangiectasia). The most feared visceral complication in ACA-positive lcSSc is isolated pulmonary arterial hypertension (PAH), occurring without significant ILD. Anti-Scl-70 (anti-topoisomerase I) is associated with diffuse SSc and ILD. Anti-RNA polymerase III is associated with diffuse SSc and renal crisis.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.