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APS vs NPSLE — SCE Rheumatology MCQ

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HardSLE & Antiphospholipid SyndromeAPS vs NPSLESCE Rheumatology

A 35-year-old woman with SLE develops an abrupt generalised seizure followed by several hours of confusion. Her extra-neurological SLE is quiescent, with stable anti-dsDNA titres and normal complement concentrations. She is persistently positive for lupus anticoagulant, high-titre IgG anticardiolipin and IgG anti-beta-2-glycoprotein I antibodies on samples obtained more than 12 weeks apart. Cerebrospinal fluid is acellular, with normal glucose and mildly elevated protein; microbiological studies are negative. MRI with diffusion-weighted imaging shows multiple punctate areas of restricted diffusion with corresponding low apparent diffusion coefficient values in several cerebral arterial territories. What is the most likely cause of her neurological presentation?

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Correct answer: A — Antiphospholipid-antibody-related thrombotic cerebrovascular disease with multifocal microinfarction

The best answer is A. The abrupt presentation, persistent high-risk triple antiphospholipid-antibody profile and multiple diffusion-restricting lesions in different arterial territories indicate acute multifocal cerebral ischaemia from antiphospholipid-antibody-related thrombosis. EULAR distinguishes these atherothrombotic manifestations from inflammatory NPSLE, for which glucocorticoids and immunosuppression are used. Inflammatory NPSLE remains an important differential for seizures and confusion, but is less likely with quiescent systemic disease, normal complement and an unequivocally ischaemic MRI pattern. Anti-ribosomal P or anti-neuronal antibody negativity would not itself exclude inflammatory NPSLE and therefore is not used as the decisive discriminator. MS overlap would usually produce non-restricting demyelinating lesions with a characteristic distribution. The acellular, microbiologically negative CSF argues against infection, while primary CNS angiitis is less likely than aPL-related thrombosis in this context.

Reference: Fanouriakis A et al. EULAR recommendations for the management of systemic lupus erythematosus: 2023 update, recommendation 6. Annals of the Rheumatic Diseases. 2024;83:15–29. https://pubmed.ncbi.nlm.nih.gov/37827694/