A 40-year-old woman has had Raynaud's phenomenon for 5 years, followed by puffy fingers (sclerodactyly), telangiectases on face and hands, and calcinosis in fingertips. ANA is positive with anti-centromere antibody (ACA). Pulmonary function tests show mild restrictive pattern. The likely diagnosis and its associated risk compared to diffuse SSc are:

• A Diffuse cutaneous systemic sclerosis (dcSSc) — high risk of renal crisis
• B Mixed connective tissue disease — requires anti-U1RNP antibody
• C Undifferentiated connective tissue disease
• D Limited cutaneous systemic sclerosis (lcSSc/CREST) — anti-centromere positive; primary risk is pulmonary arterial hypertension (PAH) ✓

Explanation

Limited cutaneous systemic sclerosis (lcSSc), formerly CREST syndrome (Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangiectasia), is characterised by anti-centromere antibodies (ACA) and skin involvement limited to distal extremities and face. The major long-term complication specific to lcSSc is pulmonary arterial hypertension (PAH), occurring in 10–15% due to progressive small pulmonary vessel involvement. Scleroderma renal crisis (SRC) is associated with dcSSc (anti-Scl-70/anti-topoisomerase I positive) and early rapidly progressive diffuse disease. ILD is common in both but more severe in dcSSc.

Reference: Neena Khanna Illustrated Synopsis of Dermatology & STD, 6th ed.

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Written and medically reviewed by the StethoPrep medical team.