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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References
Hughes EH and Dick AD (2003) The pathology and pathogenesis of retinal vasculitis. Neuropathol Appl Neurobiol 29: 325–340
Stone JH et al. (1994) Sensitivities of noninvasive tests for central nervous system vasculitis: a comparison of lumbar puncture, computed tomography, and magnetic resonance imaging. J Rheumatol 21: 1277–1282
Benseler SM et al. (2005) Angiography-negative primary central nervous system vasculitis in children: a newly recognized inflammatory central nervous system disease. Arthritis Rheum 52: 2159–2167
MacLaren K et al. (2005) Primary angiitis of the central nervous system: emerging variants. QJM 98: 643–654
Govoni M et al. (2004) Recent advances and future perspective in neuroimaging in neuropsychiatric systemic lupus erythematosus. Lupus 13: 149–158
Anderson JR (1981) Intracerebral calcification in a case of systemic lupus erythematosus with neurological manifestations. Neuropathol Appl Neurobiol 7: 161–166
Wasserman BA et al. (2001) Reliability of normal findings on MR imaging for excluding the diagnosis of vasculitis of the central nervous system. AJR Am J Roentgenol 177: 455–459
Trevisani VF et al. (2000) Cyclophosphamide versus methylprednisolone for the treatment of neuropsychiatric involvement in systemic lupus erythematosus. Cochrane Database of Systematic Reviews, Issue 3 Art. No CD002265
Barile-Fabris L et al. (2005) Controlled clinical trial of IV cyclophosphamide versus IV methylprednisolone in severe neurological manifestations in systemic lupus erythematosus. Ann Rheum Dis 64: 620–625
Boumpas DT et al. (1995) Systemic lupus erythematosus: emerging concepts. Part 1: Renal, neuropsychiatric, cardiovascular, pulmonary, and hematologic disease. Ann Intern Med 122: 940–950
West SG (2003) Central nervous system vasculitis. Curr Rheumatol Rep 5: 116–127
Euler HH et al. (1994) Treatment-free remission in severe systemic lupus erythematosus following synchronization of plasmapheresis with subsequent pulse cyclophosphamide. Arthritis Rheum 37: 1784–1794
Levy Y et al. (1999) Serologic and clinical response to treatment of systemic vasculitis and associated autoimmune disease with intravenous immunoglobulin. Int Arch Allergy Immunol 119: 231–238
Lhotta K et al. (2004) Cerebral vasculitis in a patient with hereditary complete C4 deficiency and systemic lupus erythematosus. Lupus 13: 139–141
Sherer Y et al. (1999) Successful treatment of systemic lupus erythematosus cerebritis with intravenous immunoglobulin. Clin Rheumatol 18: 170–173
Valesini G et al. (1994) Central nervous system involvement in systemic lupus erythematosus: a new therapeutic approach with intrathecal dexamethasone and methotrexate. Springer Semin Immunopathol 16: 313–321
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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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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DOI: https://doi.org/10.1038/ncprheum0337
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