抗中性粒细胞胞质抗体相关性肥厚性硬脑膜炎患者临床特点及影像学分析  

Clinical and imaging analysis of the anti-neutrophil cytoplasmic antibody associated hypertrophic pachymeningitis

作　　者：陈晓莉[1] 王群 刘佳[3] 李静[3] 张国平[2] CHEN Xiaoli;WANG Qun;LIU Jia;LI Jing;ZHANG Guoping(不详;Department of Medical Imaging Diagnosis Center,Shaanri Provincial People's Hospital,Xi'an 710068,China)

机构地区：[1]陕西省人民医院神经内科,710068 [2]陕西省人民医院医学影像诊断中心,710068 [3]中南大学湘雅医院,410008

出　　处：《中国神经免疫学和神经病学杂志》2024年第1期40-45,共6页Chinese Journal of Neuroimmunology and Neurology

摘　　要：目的分析抗中性粒细胞胞质抗体(anti-neutrophil cytoplasmic antibody,ANCA)相关性肥厚性硬脑膜炎(hypertrophic pachymeningitis,HP)患者的临床特点。方法回顾性收集2015年8月至2022年6月作者单位收治的10例ANCA相关性HP患者,其中来自中南大学湘雅医院9例,陕西省人民医院1例,男、女各5例,发病年龄43~80岁。分析患者的临床特征、影像学特点、诊治方法及疗效。结果10例患者表现为不同程度的慢性头痛或脑神经受累症状,病程1~84个月。5例患者血清髓过氧化物酶(myeloperoxidase,MPO)和核周型ANCA(perinuclear ANCA,p-ANCA)同时阳性,2例患者仅血清MPO阳性,p-ANCA和细胞质型ANCA(cytoplasmic ANCA,c-ANCA)阳性各1例,p-ANCA和c-ANCA同时阳性1例。6例视力受累,2例听力受累。增强MRI显示患者不同程度的硬脑膜增厚并强化,其中弥漫性增厚4例,局灶性增厚6例,均未累及脑实质。1例患者单用糖皮质激素治疗,1例患者行糖皮质激素联合丙球治疗,8例患者糖皮质激素联合免疫抑制剂治疗,其中1例同时使用利妥昔单抗,经治疗后患者症状均明显缓解。2例在激素和免疫抑制剂减量过程出现头痛加重,再次治疗后症状缓解。结论ANCA相关性HP病程长短不一,临床表现不典型,增强MRI显示的硬脑膜增厚部位在不同ANCA抗体阳性患者间有重叠;糖皮质激素和免疫抑制剂治疗ANCA相关性HP有效。Objective To analyze the clinical characteristics of anti-neutrophil cytoplasmic antibody(ANCA)associated hypertrophic pachymeningitis(HP).Methods From August 2015 to June 2022,10 patients with ANCA-related HP admitted to the authors'unit were retrospectively collected,including 9 cases from Xiangya Hospital of Central South University and 1 case from Shaanxi Provincial People's Hospital,5 males and 5 females,aged 43-80 years.The clinical features,imaging features,diagnosis,treatments,and efficacy of the patients were analyzed.Results Ten patients presented with varying degrees of chronic headache or cranial nerve involvement,and the course of disease ranged from 1 to 84 months.Serum myeloperoxidase(MPO)ANCA and perinuclear pattern ANCA(p-ANCA)were both positive in 5 patients,only serum MPO was positive in 2 patients,p-ANCA and cytoplasmic ANCA(c-ANCA)was positive in 1 patient separately.Six cases had visual impairment and 2 cases had hearing impairment.Enhanced MRI showed different degrees of dural thickening and enhancement,including diffuse thickening in 4 cases and focal thickening in 6 cases,without the brain parenchyma involvement.One patient was treated with glucocorticoid alone.One patient was treated with glucocorticoid combined with gamma globulin.Eight patients were treated with glucocorticoid combined with immunosuppressive agents,and 1 patient was treated with rituximab at the same time.After treatment,the symptoms of the patients were significantly relieved.Headache aggravated in 2 cases during the reduction of hormones and immunosuppressive agents,and the symptoms were relieved after re-treatment.Conclusions The course of ANCA-related HP is varying,and the clinical manifestations are not typical.The location of dural thickening on enhanced MRI overlaps between different ANCA antibody-positive patients.Glucocorticoid and immunosuppressive agents are effective in the treatment of ANCA-HP.

关 键 词：肥厚性硬脑膜炎 抗体 抗中性白细胞胞质 磁共振成像 过氧化物酶 

分 类 号：R742[医药卫生—神经病学与精神病学]