Medicine · Rheumatology (SLE, RA, Vasculitis, Crystal Arthropathies, Scleroderma)

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
Correct answer: B. Pulmonary arterial hypertension

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.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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