Anti-RNA polymerase III antibody positivity in systemic sclerosis is most closely associated with which combination of features?
- A Limited cutaneous disease with isolated pulmonary arterial hypertension
- B Diffuse cutaneous disease with severe progressive interstitial lung disease
- C Diffuse cutaneous disease, rapid skin thickening, scleroderma renal crisis, and increased risk of concurrent malignancy ✓
- D Myositis overlap with anti-PM/Scl antibodies and good prognosis
Explanation
Anti-RNA polymerase III defines a diffuse cutaneous systemic sclerosis subset with rapid onset and progression of skin thickening, high risk of scleroderma renal crisis, and a recognised association with de novo solid malignancies detected close to scleroderma onset. Anti-centromere antibodies mark limited disease with pulmonary arterial hypertension (option A), anti-topoisomerase I (anti-Scl-70) marks severe fibrotic interstitial lung disease (option B), and anti-PM/Scl marks polymyositis-scleroderma overlap (option D). Recognising these antibody-subphenotype pairings is repeatedly examined.
Reference: Kelley's Textbook of Rheumatology, 11th ed.
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