Systemic Sclerosis — SCE Rheumatology MCQ
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Correct answer: D — Limited cutaneous systemic sclerosis
Anti-centromere antibodies are most strongly associated with limited cutaneous systemic sclerosis, making E the best answer. This phenotype classically includes longstanding Raynaud phenomenon, distal skin sclerosis, telangiectasia, calcinosis and gastrointestinal dysmotility. Anti-centromere-positive limited disease generally carries a lower risk of clinically significant interstitial lung disease and scleroderma renal crisis than anti-topoisomerase I- or anti-RNA polymerase III-associated systemic sclerosis, although pulmonary hypertension remains an important screening concern. Diffuse cutaneous systemic sclerosis is more commonly associated with anti-topoisomerase I or anti-RNA polymerase III antibodies. Mixed connective tissue disease is associated with high-titre anti-U1-RNP, while dermatomyositis and systemic lupus erythematosus have different characteristic autoantibody profiles.
Reference: Binda M et al. Phenotype of diffuse cutaneous systemic sclerosis patients with positive anticentromere antibodies: a systematic literature review and meta-analysis. Seminars in Arthritis and Rheumatism. 2025;70:152606. https://pubmed.ncbi.nlm.nih.gov/39657361/