A 42-year-old woman presents with Raynaud's phenomenon, puffy fingers, telangiectasias, and mild esophageal dysmotility. ANA is positive; anti-centromere antibody is strongly positive. Anti-Scl-70 (anti-topoisomerase I) is negative. The most likely clinical course and complication to monitor for is:
- A Rapidly progressive interstitial lung disease and renal crisis
- B Pulmonary arterial hypertension (PAH) as the major vascular complication ✓
- C Scleroderma renal crisis with hypertensive emergency
- D Diffuse skin thickening progressing to trunk within 1 year
Explanation
Anti-centromere antibody is characteristic of limited cutaneous systemic sclerosis (lcSSc, formerly CREST syndrome — Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangiectasia). Limited SSc has a better prognosis with skin confined to distal extremities and face; however, pulmonary arterial hypertension (PAH) develops in 10–15% and is the leading cause of mortality. Anti-Scl-70 (anti-topoisomerase I) is associated with diffuse cutaneous SSc, which carries higher risk of ILD and scleroderma renal crisis. Annual echocardiography and right heart catheterization are essential to screen for PAH in anti-centromere positive patients.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.