A 45-year-old woman presents with Raynaud's phenomenon, dysphagia, telangiectasias, and tight skin limited to the hands and face. She is anti-centromere antibody positive. Which complication requires urgent cardiac monitoring and is the leading cause of death in this subset?

• A Renal crisis with malignant hypertension
• B Intestinal pseudo-obstruction
• C Myocarditis with complete heart block
• D Pulmonary arterial hypertension ✓

Explanation

This presentation describes limited cutaneous systemic sclerosis (lcSSc, formerly CREST syndrome). While scleroderma renal crisis is more associated with dcSSc (anti-topoisomerase I, diffuse skin), pulmonary arterial hypertension (PAH) is the leading cause of death in lcSSc associated with anti-centromere antibodies. PAH develops in 10–15% of lcSSc patients. Annual echocardiographic screening and right heart catheterization (gold standard for diagnosis) are recommended. Treatment includes endothelin receptor antagonists, PDE-5 inhibitors, and prostacyclins.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.