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NPSLE — RACP Adult Medicine MCQ

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HardRheumatologyNPSLERACP Adult Medicine

A 30-year-old woman with known SLE presents with seizures, psychosis, and focal neurological deficits (hemiparesis). MRI brain shows multifocal white matter lesions. CSF shows mild pleocytosis and elevated protein. Anti-ribosomal P antibodies are positive. C3 and C4 are low. Anti-dsDNA is rising. What is the most likely neuropsychiatric SLE manifestation?

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Correct answer: DCNS lupus (NPSLE)

Seizures, psychosis, and focal neurological deficits in active SLE (rising anti-dsDNA, low complement) with MRI white matter lesions is neuropsychiatric SLE (NPSLE). NPSLE affects ~25-75% of SLE patients. Anti-ribosomal P antibodies are associated with psychosis in SLE. Differentiate from APS-related cerebrovascular events (thrombotic) and infection (immunosuppressed). Treatment: pulse IV methylprednisolone + cyclophosphamide (or rituximab) for severe NPSLE. Anticoagulation if APS co-exists.

Reference: EULAR – 2024 – NPSLE; ACR 2024