A 60-year-old female with long-standing systemic sclerosis presents with Raynaud phenomenon, sclerodactyly, and severe dyspnea. Echocardiography suggests elevated pulmonary pressures. What autoantibody profile is most strongly associated with the CREST variant of limited cutaneous systemic sclerosis?

Answer and explanation

Correct answer: D. Anti-Centromere Antibodies (ACA)

  1. Step 1: Limited cutaneous systemic sclerosis (CREST syndrome: Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangiectasia) is strongly associated with Anti-Centromere Antibodies (ACA) in over 70% of cases, carrying a high risk of late isolated pulmonary arterial hypertension.
  2. Step 2: Consequently, the correct clinical answer is Anti-Centromere Antibodies (ACA).
Answer
Anti-Centromere Antibodies (ACA).
Why not the others: 'Anti-Scl-70 (Anti-Topoisomerase I)' is characteristic of diffuse cutaneous systemic sclerosis with rapid visceral renal and pulmonary fibrosis; 'Anti-Jo-1' is seen in polymyositis and dermatomyositis with interstitial lung disease; 'Anti-dsDNA' is highly specific for systemic lupus erythematosus (SLE).

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