A 48-year-old woman presents with episodic pallor followed by cyanosis then erythema of all fingers on cold exposure, oral mucosal telangiectasias, heartburn, and skin tightening of the fingers extending to the elbows. Anti-centromere antibodies are positive. Which complication is she MOST at risk for?

• A Pulmonary arterial hypertension ✓
• B Renal crisis (hypertensive)
• C Pulmonary fibrosis
• D Myocardial fibrosis with arrhythmia

Explanation

This patient has limited cutaneous systemic sclerosis (lcSSc, formerly CREST syndrome) with anti-centromere antibodies. The most common serious internal organ complication in lcSSc is pulmonary arterial hypertension (PAH), occurring in 15–25% and being a leading cause of death. Renal scleroderma crisis and significant pulmonary fibrosis are far more commonly associated with diffuse cutaneous SSc (dcSSc) and anti-topoisomerase I (Scl-70) antibodies.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.