中华临床免疫和变态反应杂志2017，Vol.11Issue(3)：241-246,6.DOI:10.3969∕j.issn.1673-8705.2017.03.008
嗜酸性肉芽肿性多血管炎并发中枢神经系统受累八例
Central Nervous System Involvement in Eosinophilic Granulomatosis with Polyangiitis:A Retrospective Study of 8 Patients
周佳鑫 1张上珠 1李菁 1赵丽丹 1王迁 1费允云1
作者信息
- 1. 中国医学科学院北京协和医学院北京协和医院风湿免疫科风湿免疫病学教育部重点实验室,北京100730
- 折叠
摘要
Abstract
Objective To analyze the clinical features of patients with central nervous system ( CNS) involvement in eosinophilic granulomatosis with polyangiitis ( EGPA) . Methods We retrospec-tively reviewed the clinical and laboratory data, as well as treatment and prognosis of patients with EGPA with CNS involvement in Peking Union Medical College Hospital from Jan 2000 to Jan 2017. Results Eight EGPA patients with CNS involvement were included, and the prevalence of CNS involvement in EGPA patients was 11%, with a male : female ratio=3 : 1. Four patients presented with cerebral ische-mia, 2 patients with cerebral hemorrhage, 1 patient with subarachnoid hemorrhage, 1 patient with cervical spinal cord involvement. Gastrointestinal involvement was more frequently observed in EGPA pa-tients with CNS involvement than those without. Four patients were tested positive with Anti-neutrophil cy-toplasmic antibodies. Six patients received lumber puncture, and 4 patients showed elevation of cerebro-spinal fluid pressure. One patient with subarachnoid hemorrhage manifested with bloody cerebrospinal flu-id, and the other 4 patients had elevated protein in the cerebrospinal fluid. 4 patients received methyl-prednisolone pulse therapy, 2 patients received large dose of glucocorticoids, and the other 2 patients re-ceived medium dose glucocorticoids, with cyclophosphamide as the first-line immunosuppressant. Among the 7 patients with long term follow up, 1 patient died with gastrointestinal perforation, 1 patient died with cerebral hemorrhage, the other 5 patients were clinically stable. Conclusion CNS involvement is uncom-mon in EGPA patients. EGPA patients with CNS manifestations should be aware of the relationship with the underlying disease. Achieving remission of EGPA is important in the treatment of CNS involvement.关键词
嗜酸性肉芽肿性/多血管炎/中枢神经系统Key words
eosinophilic granulomatosis with polyangiitis/central nervous system分类
医药卫生引用本文复制引用
周佳鑫,张上珠,李菁,赵丽丹,王迁,费允云..嗜酸性肉芽肿性多血管炎并发中枢神经系统受累八例[J].中华临床免疫和变态反应杂志,2017,11(3):241-246,6.
