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Antiphospholipid Syndrome — SCE Rheumatology MCQ

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ModerateSLE & Antiphospholipid SyndromeAntiphospholipid SyndromeSCE Rheumatology

A woman with established systemic lupus erythematosus has the following results on two occasions 12 weeks apart: anti-dsDNA positive, anti-Sm positive, anti-Ro positive, anti-La negative, lupus anticoagulant positive, high-titre IgG anti-β2-glycoprotein I positive and anticardiolipin negative. She has no previous thrombosis or pregnancy morbidity. Which outcome is most strongly predicted by the antiphospholipid-antibody component of this profile?

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Correct answer: AArterial or venous thrombosis

The correct answer is A. Persistent lupus anticoagulant together with high-titre anti-β2-glycoprotein I constitutes a high-risk antiphospholipid-antibody profile, despite negative anticardiolipin antibodies. Such profiles are associated with arterial and venous thrombosis and adverse obstetric outcomes. This is double, not triple, aPL positivity. Anti-dsDNA may correlate with lupus activity and nephritis, but positivity alone does not establish active renal disease. Anti-Ro is associated with subacute cutaneous lupus and creates a risk of neonatal lupus, including fetal congenital heart block, during pregnancy; it does not supersede the thrombotic implication of the specified aPL profile. Anti-Ro positivity alone also does not diagnose secondary Sjögren syndrome.

Reference: Tektonidou MG et al. EULAR recommendations for the management of antiphospholipid syndrome in adults. Annals of the Rheumatic Diseases. 2019;78:1296–1304. https://pubmed.ncbi.nlm.nih.gov/31092409/