A 60-year-old woman presents with progressive dysphagia, Raynaud's phenomenon, sclerodactyly, and telangiectasias. ANA is positive with an anticentromere antibody pattern. What is the pulmonary complication she is most at risk for?
- A Interstitial lung disease with fibrosis
- B Pulmonary arterial hypertension ✓
- C Obliterative bronchiolitis
- D Pleural effusion
Explanation
This patient has limited cutaneous systemic sclerosis (lcSSc, formerly CREST syndrome) characterised by anticentromere antibody positivity. While diffuse cutaneous SSc (anti-Scl-70/anti-topoisomerase-I positive) is associated predominantly with interstitial lung disease and pulmonary fibrosis, lcSSc/anticentromere-positive patients are at greatest risk for pulmonary arterial hypertension (PAH). Annual echocardiographic screening is recommended in lcSSc for early PAH detection.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.