以肥厚性硬脑膜炎为主要表现的肉芽肿性多血管炎临床病例分析  

Clinical analysis of 19 cases with granulomatosis with polyangiitis complicated with hypertrophic cranial pachymeningitis

作　　者：苟丽娟[1] 吴庆军[2] 王立[2] 钱敏[3] 陈华[2] 郑文洁[2] 赵岩[2] 张奉春[2] 曾小峰[2] Gou Lijuan;Wu Qingjun;Wang Li;Qian Min;Chen Hua;Zheng Wenjie;Zhao Yan;Zhang Fengchun;Zeng Xiaofeng(Department of Pediatrics,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of Rheumatology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of Neurology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China)

机构地区：[1]中国医学科学院,北京协和医学院,北京协和医院儿科,100730 [2]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,100730 [3]中国医学科学院,北京协和医学院,北京协和医院神经内科,100730

出　　处：《中华风湿病学杂志》2020年第1期13-17,共5页Chinese Journal of Rheumatology

摘　　要：目的探讨肉芽肿性多血管炎(GPA)继发肥厚性硬脑膜炎(HCP)的临床特点。方法回顾性分析北京协和医院2004—2018年收治的GPA继发HCP病例资料的特点。结果①GPA患者315例,19例继发HCP,占6.0%;②男性12例,女性7例;年龄19~64岁,中位年龄57岁。③神经系统表现:19例均有头痛,16例颅神经受累。受累部位:额部8例,颞部8例,颅底8例(鞍旁4例,其中海绵窦3例,眶尖2例),小脑幕6例,大脑镰2例,顶部1例,枕部1例,1例合并硬脊膜炎。④系统表现:发热10例,体质量下降8例,肺部受累4例,肾脏受累3例,16例鼻窦炎,10例中耳炎,16例局限型GPA。⑤15例ANCA抗体阳性,8例蛋白酶3(PR3)-ANCA阳性,6例髓过氧化物酶(MPO)-ANCA阳性。⑥16例行腰椎穿刺检查:脑脊液压力9例升高、5例正常、2例降低;脑脊液蛋白升高10例。⑦15例(78.9%)伯明翰系统性血管炎评分(BVAS)>15分。⑧19例均使用糖皮质激素、免疫抑制剂治疗,其中12例行甲泼尼龙冲击治疗,12例鞘内注射地塞米松(或+甲氨蝶呤),19例病情均缓解。结论HCP是GPA少见且严重的表现,主要表现为颅高压和颅神经受累,多见于局限型GPA患者,常伴有全身疾病的活动,需积极治疗。Objective To discuss the clinical features and treatment of 19 patients with granulomatosis with polyangiitis(GPA)complicated with hypertrophic cranial pachymeningitis(HCP).Methods The clinical features of 19 patients diagnosed with GPA complicated with HCP in Peking Union Medical College Hospital were retrospectively analyzed.Results Among the 315 patients with GPA,19(12 males,7 females,with)were diagnosed with HCP at Peking Union Medical College Hospital.The median age was 57(19-64)years.In the neurological manifestations per se,all patients had headache,16 patients had cerebral involvement,which included 8 cases at the frontal area,8 cases at the temporal area,8 cases at the skull base area(4 cases with parasellar involvements including 3 cases with cavernous sinus involvement and 2 cases with orbital involvement),6 cases of tentorium involvement,2 cases of cerebral palsy,1 case of calvarium,1 case of occipital,and 1 case with combined spinal pachymeningitis,respectively.In systemic manifestations,10 patients had fever,8 patients had weight lose,4 patients had lung involvement,3 patients had kidney involvement,16 patients had nasosinusitis,10 patients had tympanitis,and 16 patients had localized GPA.The laboratory tests showed that 15 patients had positive anti-neutrophil cytoplasmic antibodies(ANCA),including 8 cases with positive proteinase 3(PR3)-ANCA and 6 cases with positive myeloperoxidase(MPO)-ANCA.Sixteen patients had lumbar puncture examination,9 cases had elevated cerebrospinal pressure,10 cases had elevated level of protein in cerebrospinal fluid.Nineteen patients were treated with glucocorticoids(12 patients accepted pulse therapy)and immunosuppressive agents.Twelve patients were treated with intrathecal injections of dexamethasone combined with or without Methotrexate(MTX).All 19 patients were improved.Conclusion HCP,as a rare but serious manifestation of GPA,is not rare in active cases and should be intensively treated.

关 键 词：血管炎 中枢神经系统 肥厚性硬脑膜炎 

分 类 号：R593[医药卫生—内科学] R74[医药卫生—临床医学]