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SLE — SCE Rheumatology MCQ

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HardSLE & Antiphospholipid SyndromeSLESCE Rheumatology

A 55-year-old woman with systemic lupus erythematosus develops progressive executive dysfunction, personality change and two generalised tonic-clonic seizures over 3 months. She has no focal neurological deficit. Complement C3/C4 concentrations have fallen and anti-dsDNA titres have risen. Antiphospholipid antibody testing is negative. Brain MRI shows scattered punctate deep white-matter FLAIR hyperintensities and mild generalised cerebral volume loss, without diffusion restriction, contrast enhancement or a mass lesion. CSF has normal cell count, protein and glucose, with negative microbiological studies and JC-virus PCR. EEG shows diffuse slowing without an epileptiform focus. Serum anti-ribosomal P antibodies are positive. What is the most likely diagnosis?

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Correct answer: E — Diffuse neuropsychiatric systemic lupus erythematosus

This is diffuse neuropsychiatric SLE (NPSLE), with severe cognitive dysfunction and seizures occurring alongside serological lupus activity after major alternative causes have been investigated. NPSLE is an attribution diagnosis: compatible syndromes must be assessed using the same neurological work-up as in patients without SLE, then attributed to lupus only after infection, structural disease, thrombosis, drug toxicity and other neurological disorders are considered. Anti-ribosomal P positivity is supportive at most; it is neither sensitive nor specific enough to establish NPSLE independently. Alzheimer disease is usually more insidious and would not readily explain the seizures and concurrent lupus activity. PML usually causes progressive focal deficits with characteristic asymmetric white-matter lesions; negative CSF JC-virus PCR and the imaging pattern argue against it. CNS lymphoma generally produces enhancing focal lesions or mass effect.

Reference: Bertsias G, et al. EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations. Ann Rheum Dis. 2010;69:2074-2082. https://pubmed.ncbi.nlm.nih.gov/20724309/