髓过氧化物酶抗中性粒细胞胞质抗体阳性的肥厚性硬脑膜炎临床分析  

Clinical analysis of MPO-ANCA associated hypertrophic pachymeningitis

作　　者：周珂珂 蒋玙姝 贾亚珍 金珂 秦灵芝 马伟锋 张涛 张梦歌 李玮 ZHOU Keke;JIANG Yushu;JIA Yazhen;JIN Ke;QIN Lingzhi;MA Weifeng;ZHANG Tao;ZHANG Mengge;LI Wei(People’s Hospital of Zhengzhou University/People’s Hospital of Henan Province,Zhengzhou 450003,China)

机构地区：[1]郑州大学人民医院河南省人民医院,河南郑州450003

出　　处：《中国实用神经疾病杂志》2021年第8期664-668,共5页Chinese Journal of Practical Nervous Diseases

基　　金：2018年度河南省医学科技攻关计划项目(编号:SBGJ2018077)。

摘　　要：目的探讨髓过氧化物酶抗中性粒细胞胞质抗体阳性(MPO-ANCA)的肥厚性硬脑膜炎(hypertrophic cranial pachymeningitis,HCP)的临床特点及诊治方法。方法回顾性分析2018—2020年河南省人民医院收治的7例MPO-ANCA阳性的HCP笔者的临床资料。结果7例患者中男6例,女1例,平均发病年龄63岁。所有患者以头痛起病,5例脑神经受累,脑实质受累1例,中耳炎2例,乳突炎2例,均无肺肾损害。初诊时均有C反应蛋白升高、红细胞沉降率增快、MPO-ANCA阳性、PR3-ANCA阴性,6例脑脊液蛋白升高。所有患者有硬脑膜的增厚并强化。糖皮质激素治疗症状改善明显,3例初始单用激素治疗停药或减量时复发,再次使用激素并联合免疫抑制剂后2例未复发;2例初始使用激素联合免疫抑制剂治疗,病情控制较好,均未复发;1例拒绝激素治疗;1例初始单用激素治疗后症状改善明显,因死亡随访终止。结论MPO-ANCA相关HCP是HCP的一种特殊类型,对于慢性头痛伴脑神经损害的老年患者,MPO-ANCA阳性时应怀疑HCP可能;无病理诊断支持时,头颅MRI增强可协助诊断,激素联合免疫抑制剂治疗效果较好。Objective To explore the clinical features,diagnosis and treatment methods of MPO-ANCA associated with hypertrophic pachymeningitis.Methods The clinical data of 7 MPO-ANCA patients were retrospectively analyzed who were admitted to Henan Provincial People’s Hospital from 2018 to 2020.Results All these 7 patients,6 males and 1 female with an average age of onset of 63 years old,had headache onset.Five patients demonstrated cranial nerve involvement,2 patients demonstrated cranial otitis media,and 2 patients demonstrated cranial mastoiditis.None of them had lung or kidney damage.At the preliminary visit,C-reactive protein increased,erythrocyte sedimentation rate increased,MPO-ANCA was positive,PR3-ANCA was negative,and all patients had CSF protein increased.All patients have increasing thickness and strength of the dura mater.Glucocorticoid treatment is considered to ameliorate the symptoms.Three patients were initially treated with hormones alone,and the disease relapsed when the medication was stopped or reduced,and then used hormones combined with immunosuppressive agents,of which 2 patients did not relapse.Two patients were initially treated with hormones combined with immunosuppressive agents,their condition was well controlled,and none of them relapsed.One patient refused hormone therapy.In one case,symptoms improved significantly after initial hormone therapy alone,and follow-up was terminated due to death.Conclusion MPO-ANCA related HCP is a special type of HCP.For elderly patients with chronic headache and cranial nerve damage,when MPO-ANCA is positive,HCP should be taken into consideration.When there is no pathological diagnosis support,enhanced MRI of the head can assist diagnosis.Hormone combined with immunosuppressant therapy has better effect.

关 键 词：肥厚性硬脑膜炎 髓过氧化物酶抗中性粒细胞胞质抗体 脑神经损伤 慢性头痛 

分 类 号：R512.3[医药卫生—内科学]