Tumor-like Cerebral Perivasculitis in a Pediatric Patient with Systemic Lupus Erythematosus
Article Outline
Nervous system manifestations are present in up to 70% of patients with systemic lupus erythematosus (SLE). The spectrum of clinical symptoms varies widely, from severe, life-threatening symptoms at presentation, such as transverse myelitis, to symptoms of more subtle and subclinical abnormalities of neurocognitive function. We report the case of a 14-year-old patient with SLE and lupus nephritis under regular steroid therapy, who had a sudden onset of consciousness change. Brain magnetic resonance imaging showed a huge mass lesion. After surgical decompression and corticosteroid pulse therapy, the patient's neurologic symptoms improved dramatically. Brain biopsy revealed perivasculitis of the brain with marked perivascular infiltration of eosinophils, macrophages, and neutrophils. Microhemorrhage was also evident. The patient recovered without obvious neurologic sequelae.
Key Words: central nervous system , magnetic resonance imaging , systemic lupus erythematosus
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References
- Cognitive impairment in patients with systemic lupus erythematous . J Rheumatol . 1992;19:562–567
- . Controversies in lupus: nervous system involvement . Ann Rheum Dis . 1995;54:159–167
- . The American College of Rheumatology nomenclature and case definitions for neuropsychiatric syndromes . Arthritis Rheum . 1999;42:599–608
- . Central nervous system lupus: a clinical approach to therapy . Lupus . 2003;12:935–942
- . CNS involvement in systemic lupus erythematosus: a case with remarkable histopathological findings . Ann Rheum Dis . 2001;60:299–300
- . Lupus and the nervous system . Lupus . 2003;12:871
- The ACR nomenclature for CNS lupus revisited . Lupus . 2003;12:872–876
- . The neuropathology and pathogenesis of systemic lupus erythematosus . Neuropathol Appl Neurobiol . 2002;28:173–189
- Neuropsychiatric manifestations in systemic lupus erythematosus: prevalence and association with antiphospholipid antibodies . J Rheumatol . 2003;30:985–992
- Neuropsychiatric manifestations in pediatric systemic lupus erythematosus and association with antiphospholipid antibodies . J Rheumatol . 2006;33:1873–1877
- . Lupus transverse myelopathy: better outcome with early recognition and aggressive high-dose intravenous corticosteroid pulse treatment . J Neurol . 1995;242:326–331
- Favorable response to intravenous methylprednisolone and cyclophosphamide in children with severe neuropsychiatric lupus . J Rheumatol . 1999;26:432–439
- . Quantitative magnetic resonance imaging in neuropsychiatric systemic lupus erythematosus . Lupus . 2003;12:897–902
PII: S1607-551X(08)70121-9
doi:10.1016/S1607-551X(08)70121-9
© 2008 Elsevier. Published by Elsevier Inc. All rights reserved.
