Nikolov Nikolay P, Smith Janine A, Patronas Nicholas J, Illei Gabor G
National Institute of Dental and Craniofacial Research, Gene Therapy and Therapeutics Branch, NIH, Bethesda, MD 20892, USA.
Nat Clin Pract Rheumatol. 2006 Nov;2(11):627-33; quiz 634. doi: 10.1038/ncprheum0337.
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.
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.
Vasculitis of the central nervous system associated with systemic lupus erythematosus.
Intravenous methylprednisolone 1,000 mg/day for 3 days, one dose of intravenous pulse cyclophosphamide 750 mg/m(2), 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.
一名23岁的白人女性，有3年系统性红斑狼疮病史、15个月狼疮性肾炎和视网膜血管炎病史，在接受中等剂量皮质类固醇治疗期间，因铜绿假单胞菌肺炎使用抗生素治疗成功。尽管她的肺炎有所改善，但她出现了精神状态的急性变化，并迅速发展为伴有昏迷的脑病。
体格检查、眼底检查、代谢异常的实验室检查、脑脊液分析、微生物学和血清学检测、脑电图、IgM和IgG抗心磷脂抗体检测、神经影像学检查，包括脑部CT以及钆对比前后的T1加权磁共振成像（MRI），还有液体衰减反转恢复序列MRI。
与系统性红斑狼疮相关的中枢神经系统血管炎。
静脉注射甲泼尼龙1000毫克/天，共3天；静脉注射一次脉冲环磷酰胺750毫克/平方米；静脉注射免疫球蛋白400毫克/千克/天，共4天；隔天进行血浆置换，共五个周期；泼尼松40毫克/天。她继续每月静脉注射脉冲环磷酰胺和静脉注射脉冲甲泼尼龙6个月，随后在2年中每3个月进行维持输注。泼尼松在18个月内逐渐减量。由于骨髓耐受性差，环磷酰胺在2年后停用，并被霉酚酸酯3000毫克/天和环孢素每日两次、每次50毫克替代。
