A 55-year-old woman with long-standing seropositive rheumatoid arthritis presents with Raynaud phenomenon, dysphagia, skin tightening over the hands, and telangiectasias. Anti-centromere antibody is positive. This antibody is most strongly associated with:
- A Diffuse cutaneous systemic sclerosis
- B Systemic lupus erythematosus
- C Polymyositis
- D Limited cutaneous systemic sclerosis (CREST syndrome) ✓
Explanation
Anti-centromere antibody is the serologic marker most strongly associated with limited cutaneous systemic sclerosis (lcSSc), formerly called CREST syndrome (Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangiectasias). In lcSSc, skin involvement is limited to distal extremities and the face, and pulmonary arterial hypertension is the major life-threatening complication rather than interstitial lung disease. Anti-Scl-70 (anti-topoisomerase I) is associated with diffuse cutaneous SSc and a greater risk of ILD.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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