髓过氧化物酶抗中性粒细胞胞质抗体相关肥厚性硬膜炎四例并文献复习  

A report of 4 cases of myeloperoxidase antineutrophil cytoplasmic antibody associated hypertrophic pachymeningitis with literature review

作　　者：赵震 关文娟[1] 刘升云[1] 张磊[1] 贺玉杰[1] 郭金燕[1] 苏静波 余祖江[1] 阚全程[1] Zhao Zhen;Guan Wenjuan;Liu Shengyun;Zhang Lei;He Yujie;Guo Jinyan;Su Jingbo;Yu Zujiang;Kan Quancheng(Department of Rheumatology,the First Affiliated Hospital of Zhengzhou University,Henan 450052,Chin)

机构地区：[1]郑州大学第一附属医院风湿免疫科,450052

出　　处：《中华风湿病学杂志》2018年第7期467-471,共5页Chinese Journal of Rheumatology

摘　　要：目的探讨髓过氧化物酶抗中性粒细胞胞质抗体（MPO-ANCA）相关肥厚性硬膜炎（HP）的临床特征。方法对我院诊治的4例MPO-ANCA相关HP患者的临床资料进行回顾性分析并复习相关文献。结果4例男性患者，发病年龄44-66岁。临床上主要表现为不同程度的头痛及多发颅神经麻痹。所有患者病情活动时均出现核周型（p）-ANCA阳性、MPO-ANCA滴度和炎症指标升高，而肾功能、胞质型（c）-ANCA和蛋白酶3（PR3）-ANCA正常。头颅增强MRI均显示硬脑膜明显增厚伴强化以及鼻窦炎或鼻窦内肿物形成。4例患者均对糖皮质激素反应敏感，3例患者激素减量时症状复发，激素加量及加用免疫抑制剂后病情控制良好。病情缓解期p-ANCA恢复阴性，MPO-ANCA和炎症指标降至正常且MRI上硬膜厚度有所减轻。结论MPO-ANCA相关HP是ANCA相关血管炎（AAV）中枢神经系统受累的一种类型，其较多累及上呼吸道，较少进展为全身系统型AAV，对临床上以头痛和多发性颅神经麻痹的中老年患者要考虑到本病的可能，增强MRI为诊断首选，必要时做硬膜活检。ObjectiveTo investigate the clinical features of myeloperoxidase antineutrophil cytoplasmic antibody （MPO-ANCA） associated hypertrophic pachymeningitis （HP）.MethodsClinical data of 4 casesdiagnosed with MPO-ANCA vasculitis complicated with HP in our hospital were analyzed retrospectively and the related literaturewere reviewed.ResultsFour male patients with an age range from 44 to 66 years were diagnosed with ANCA-associated HP. The main clinical manifestations included headache and with- various degree ofmultiple cranial paralysis. During active phase of the disease, all patients showed perinu- clear（p）-ANCA positive, elevated levels of inflammatory biomarkers and titers of MPO-ANCA, whereas renal function, cytoplasmic （c）-ANCA and protease 3 （PR3）-ANCA were negative. Contrast-enhanced cranial ma- gnetic resonance imaging （MRI） scan showed obviously thickened dura mater and sinusitis or mass in paranasal sinus. Four patients were sensitive to glucocorticoid. Three patients had a relapse during glucocorti- coid tapering and were undercontrol when the dosage of glucocorticoid was increased and immunosuppressive agents were added. Levels of inflammatory biomarkers, titers of MPO-ANCA and p-ANCA recovered to normal, and the dural thickness on MRI was reduced in the remission stage.ConclusionMPO-ANCA associated HP is a type of central nervous system involvement in ANCA associated vasculitis （AAV）. It involves the upper respiratory tract more frequently, and less frequently progresses to systemic AAV. This should be taken into consideration when middle-aged and elderly patients presented with headache and multiple cranial neuropathies. Enhanced MRI is the preferred examination for diagnosis, and dural biopsy should be done when necessary.

关 键 词：髓过氧化物酶 抗中性粒细胞胞浆抗体 硬膜炎 肥厚 肉芽肿 多血管炎 

分 类 号：R741[医药卫生—神经病学与精神病学]