Fulminant onset of neuropsychiatric symptoms as first manifestation of neuropsychiatric systemic lupus erythematosus (NPSLE) is rare and diagnosis is difficult if only one organ is involved. Here, we report the case of a previously healthy woman who presented with a clinical syndrome most compatible with acute onset of NPSLE. However, American College of Rheumatology (ACR) criteria were not sufficiently met. Brain biopsy showed an autoimmune complex vasculitis consistent with central nervous system (CNS) lupus. Because the prognosis of SLE-related CNS involvement is poor, aggressive immunosuppressive treatment was initiated using methylprednisolone in combination with cyclophosphamide.
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