Scleroderma (systemic sclerosis) is characterised by fibrosis. Anti-topoisomerase I (Scl-70) antibodies are associated with which subtype, and anti-centromere antibodies with which subtype?

• A Anti-Scl-70: limited cutaneous SSc (CREST); anti-centromere: diffuse SSc
• B Anti-Scl-70: primary Sjögren syndrome; anti-centromere: mixed connective tissue disease
• C Anti-Scl-70: morphoea (localised scleroderma); anti-centromere: systemic sclerosis with myositis
• D Anti-Scl-70: diffuse cutaneous SSc (severe fibrosis, ILD, renal crisis); anti-centromere: limited cutaneous SSc (CREST syndrome) ✓

Explanation

Anti-topoisomerase I (anti-Scl-70) antibodies are a marker of diffuse cutaneous systemic sclerosis (dcSSc), which carries higher risk of interstitial lung disease, renal crisis, and extensive skin fibrosis. Anti-centromere antibodies (ACA) are associated with limited cutaneous SSc (lcSSc), previously CREST syndrome (Calcinosis, Raynaud, oEsophageal dysmotility, Sclerodactyly, Telangiectasia), which has a more indolent course but risk of pulmonary arterial hypertension.

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

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