A 45-year-old woman presents with Raynaud's phenomenon, dysphagia for solids, salt-and-pepper skin changes, calcinosis, and telangiectasias. ANA is positive with anti-centromere antibody pattern. The most likely diagnosis and its most serious pulmonary complication are:

• A Diffuse cutaneous systemic sclerosis (dcSSc) — interstitial lung disease
• B Mixed connective tissue disease — pleuritis
• C SLE — lupus pneumonitis
• D Limited cutaneous systemic sclerosis (lcSSc) — pulmonary arterial hypertension ✓

Explanation

CREST syndrome features (Calcinosis, Raynaud's, oEsophageal dysmotility, Sclerodactyly, Telangiectasias) with anti-centromere antibodies define limited cutaneous systemic sclerosis. The most feared pulmonary complication in lcSSc is pulmonary arterial hypertension (PAH), which occurs in ~15% of cases and is the leading cause of death. Diffuse SSc (with anti-Scl-70 antibodies) is more associated with interstitial lung disease and renal crisis.

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

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Written and medically reviewed by the StethoPrep medical team.