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SSc Autoantibody Organ Prediction — SCE Rheumatology MCQ

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ModerateSystemic SclerosisSSc Autoantibody Organ PredictionSCE Rheumatology

A 45-year-old woman has systemic sclerosis and is positive for anti-topoisomerase I (Scl-70) antibodies. Systemic sclerosis-specific autoantibodies are used alongside cutaneous subset and disease duration to stratify the risk of major complications. Which set of autoantibody associations is most accurate?

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Correct answer: B — Anti-centromere—PAH; anti-topoisomerase I—ILD; anti-RNA polymerase III—renal crisis and malignancy near disease onset

Explanation lettering: D = shown as A · E = shown as D · A = shown as E

B is correct. Anti-centromere antibodies are associated with limited cutaneous SSc, relatively little ILD and an increased risk of pulmonary arterial hypertension. Anti-topoisomerase I (Scl-70) predicts a substantially increased risk of SSc-associated ILD, including clinically significant pulmonary fibrosis. Anti-RNA polymerase III is strongly associated with rapidly progressive diffuse skin disease and scleroderma renal crisis; it also identifies a cancer-associated phenotype in which malignancy is temporally clustered around SSc onset. These are risk associations rather than deterministic predictions, so screening for organ involvement remains necessary in all patients. The other options incorrectly reassign ILD, PAH or renal-crisis risk among the antibody groups, or incorrectly suggest that anti-RNA polymerase III reduces malignancy risk.

Reference: Komura K. The clinical utility of autoantibodies in systemic sclerosis: a review with a focus on cohort differences and standardization. Frontiers in Immunology. 2025. https://pubmed.ncbi.nlm.nih.gov/40676730/