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Diffuse NPSLE — SCE Rheumatology MCQ

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HardSLE & Antiphospholipid SyndromeDiffuse NPSLESCE Rheumatology

A 40-year-old woman with established systemic lupus erythematosus develops an 8-month history of progressive memory impairment, slowed information processing and personality change. Formal neuropsychological assessment confirms deficits in attention, executive function and verbal learning. She has no persistent low mood, anhedonia, psychosis, delirium or focal neurological signs. MRI shows generalised cerebral volume loss without infarction, significant white-matter ischaemic disease or disproportionate ventricular enlargement. CSF is acellular with normal protein, and infectious, endocrine, metabolic and drug-related causes have been excluded. Antiphospholipid antibody testing is repeatedly negative. Anti-ribosomal P antibodies are present, although anti-dsDNA titres and complement remain stable. Which diagnosis best classifies this presentation?

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Correct answer: E — Cognitive dysfunction attributable to neuropsychiatric SLE

This is **cognitive dysfunction attributable to neuropsychiatric SLE**, a diffuse NPSLE phenotype. The decisive feature is objective impairment across several cognitive domains without focal neurological findings or an alternative metabolic, infectious, psychiatric or structural explanation. Normal complement, stable anti-dsDNA and bland CSF do not exclude NPSLE. Anti-ribosomal P positivity is at most supportive: it is associated more consistently with psychiatric manifestations and has insufficient diagnostic accuracy to establish cognitive NPSLE independently. Mood disorder is excluded by the absence of a depressive syndrome. Young-onset Alzheimer disease is less likely given the clinical context and completed exclusion assessment. Normal-pressure hydrocephalus would require disproportionate ventriculomegaly and typically gait or urinary disturbance. Cerebrovascular NPSLE usually produces infarcts, focal or stepwise deficits, and is particularly associated with antiphospholipid antibodies.

Reference: Bertsias GK et al. EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations, sections on diagnosis and cognitive dysfunction, 2010. https://pubmed.ncbi.nlm.nih.gov/20724309/