嗜酸性肉芽肿性多血管炎并发中枢神经系统受累八例

摘要

目的 分析嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiitis,EGPA)并发中枢神经系统受累患者的临床特点.方法 回顾性分析2000年1月至2017年1月北京协和医院诊治的EGPA并发中枢神经系统受累患者的临床、 实验室检查资料以及治疗和预后.结果 73例EGPA患者中,66例资料齐全;8例(11%)并发中枢神经系统受累,男:女=3:1,其中4例脑梗死或缺血、2例脑出血、1例蛛网膜下腔出血、1例颈髓受累.与未并发中枢神经系统受累EGPA患者相比,中枢神经系统受累患者更容易出现消化道受累(6/8 vs.10/58,P=0.002).8例中枢神经系统受累患者中,4例抗中性粒细胞胞浆抗体阳性;6例行腰椎穿刺术检查,其中4例脑脊液压力升高,1例蛛网膜下腔出血患者表现为血性脑脊液,4例脑脊液蛋白升高;4例接受糖皮质激素冲击治疗,2例接受大剂量糖皮质激素治疗,另有2例接受中等剂量糖皮质激素治疗,免疫抑制剂首选环磷酰胺;5例经长期随访病情稳定,1例因消化道穿孔死亡,1例因脑出血死亡,1例失访.结论 中枢神经系统受累为EGPA的少见并发症,EGPA患者出现中枢神经系统症状时应警惕原发病累及中枢神经系统的可能,积极控制原发病为治疗的基础.%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.