A 55-year-old woman with limited cutaneous systemic sclerosis (lcSSc) develops worsening exertional dyspnea. She has anti-centromere antibodies, calcinosis, Raynaud's, and telangiectasia (CREST). Echo suggests elevated RVSP. What is the most common cause of death in lcSSc?

• A Scleroderma renal crisis
• B Pulmonary arterial hypertension ✓
• C Interstitial lung disease with fibrosis
• D Malabsorption and nutritional failure

Explanation

In limited cutaneous systemic sclerosis (lcSSc/CREST), pulmonary arterial hypertension (PAH) is the leading cause of death, occurring late in disease course (10–20 years after Raynaud's onset). Anti-centromere antibodies are strongly associated with PAH risk. Annual echocardiographic screening is recommended. In diffuse cutaneous SSc (dcSSc), interstitial lung disease and scleroderma renal crisis are more prominent early causes of morbidity and death.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.