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Diagnosis and treatment of vasculitis of the central nervous system in a patient with systemic lupus erythematosus

Abstract

Background A 23-year-old white woman with a 3-year history of systemic lupus erythematosus and a 15-month history of lupus nephritis and retinal vasculitis was successfully treated with antibiotics for Pseudomonas aeruginosa pneumonia while on moderate doses of corticosteroids. Even though her pneumonia had improved, she developed acute changes in her mental status that rapidly progressed to encephalopathy with coma.

Investigations Physical examination, fundoscopic examination, laboratory tests for metabolic abnormalities, cerebrospinal fluid analysis, microbiology and serologic testing, electroencephalogram, tests for IgM and IgG anticardiolipin antibodies, neuroimaging including CT of the brain and T1-weighted MRI before and after gadolinium contrast, and flow-attenuated inversion recovery MRI.

Diagnosis Vasculitis of the central nervous system associated with systemic lupus erythematosus.

Management Intravenous methylprednisolone 1,000 mg/day for 3 days, one dose of intravenous pulse cyclophosphamide 750 mg/m2, intravenous immunoglobulin 400 mg/kg/day for 4 days, plasmapheresis on alternate days for five cycles, and prednisone 40 mg/day. She continued monthly doses of intravenous pulse cyclophosphamide and intravenous pulse methylprednisolone for 6 months, followed by maintenance infusions every 3 months over 2 years. Prednisone was tapered over 18 months. Cyclophosphamide was discontinued after 2 years because of poor bone-marrow tolerance, and was replaced with mycophenolate mofetil 3,000 mg/ day and ciclosporin 50 mg twice daily.

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Figure 1: Ophthalmologic examinations at initial presentation and at 10-month follow-up.
Figure 2: Diagnostic approach to CNS vasculitis.
Figure 3: CT scan of the brain, showing multiple punctate lesions (white arrows) in the basal ganglia and in the white matter of both cerebral hemispheres.
Figure 4: T1-weighted MRI of the brain after gadolinium contrast, showing three hyperintense lesions.

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Acknowledgements

We would like to thank Dr Daniel L Kastner and Dr Paolo A Muraro for their critical review of the manuscript and invaluable suggestions.

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Correspondence to Gabor G Illei.

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The authors declare no competing financial interests.

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Nikolov, N., Smith, J., Patronas, N. et al. Diagnosis and treatment of vasculitis of the central nervous system in a patient with systemic lupus erythematosus. Nat Rev Rheumatol 2, 627–633 (2006). https://doi.org/10.1038/ncprheum0337

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