髓过氧化物酶抗中性粒细胞胞质抗体相关肥厚性硬膜炎15例临床分析  

Clinical analysis of 15 cases with myeloperoxidase antineutrophil cytoplasmic antibody associated hypertrophic pachymeningitis

作　　者：关文娟[1] 丁艳霞[1] 刘丽君[1] 李伟[1] 景黎君[2] 张欣[1] 张丽娟 李慧[1] 程书华[1] 刘升云[1] Guan Wenjuan;Ding Yanxia;Liu Lijun;Li Wei;Jing Lijun;Zhang Xin;Zhang Lijuan;Li Hui;Cheng Shuhua;Liu Shengyun(Department of Rheumatology and Immunology,First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China;Department of Neurology,First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院风湿免疫科,郑州450052 [2]郑州大学第一附属医院神经内科,郑州450052

出　　处：《中华内科杂志》2022年第5期565-569,共5页Chinese Journal of Internal Medicine

摘　　要：总结并分析髓过氧化物酶(MPO)-抗中性粒细胞胞质抗体(ANCA)相关肥厚性硬膜炎(HP)的临床特点。回顾性分析15例MPO-ANCA相关HP患者的临床资料,男性9例,女性6例,年龄(58±8)岁。15例患者均有不同程度的慢性头痛。增强磁共振成像(MRI)提示有不同程度的硬脑膜/脊膜增厚并强化,均未累及脑实质。9例患者有多对颅神经损害,以Ⅴ、Ⅷ颅神经受累最为常见,主要表现为面部疼痛(4例)、听力下降(3例)、耳鸣(1例)。2例患者同时合并肥厚性硬脊膜炎(HSP);4例患者出现肺部病变。15例患者血清MPO和核周型ANCA(pANCA)同时阳性;2例患者同时合并IgG 4 阳性。14例患者红细胞沉降率(25~116 mm/1h)和C反应蛋白(29.02~146.00 mg/L)显著升高,9例患者颅内压[180~235 mmH 2O(1 mmH 2O=0.009 8 kPa)]和脑脊液中蛋白升高(457.6~3 710.0 mg/L)。6例患者单用糖皮质激素(泼尼松,20~60 mg/d)治疗,9例患者糖皮质激素(泼尼松20~60 mg/d)联合免疫抑制剂(甲氨蝶呤15 mg/周或口服环磷酰胺100 mg/d)治疗,症状均明显缓解。MPO-ANCA相关HP是ANCA相关血管炎(AAV)中枢神经受累的一种特殊类型,较少出现肺、肾等其他系统受累,糖皮质激素和免疫抑制剂治疗有效。在不明原因HP的鉴别诊断时,需常规检测ANCA和IgG 4,排除AAV或IgG 4相关性疾病并发HP的可能性。To study the clinical features of myeloperoxidase(MPO)antineutrophil cytoplasmic antibody(ANCA)associated hypertrophic pachymeningitis(HP).Clinical data of 15 cases diagnosed with MPO-ANCA vasculitis complicated with HP were retrospectively analyzed.Nine cases were males and the other 6 were females,with an average age of(58±8)years.All cases presented with chronic headache.Contrast-enhanced magnetic resonance imaging(MRI)scan showed local or diffused thickening of cerebral and/or spinal dura matter while brain parenchyma were normal.Nine cases developed multiple cranial nerve paralysis,with trigeminal nerve and auditory nerve involved most commonly.The main clinical manifestations were facial pain,hearing loss and tinnitus.Two cases were complicated with hypertrophic spinal pachymeningitis(HSP)and 4 cases were complicated with pulmonary diseases.Positive serum perinuclear pattern ANCA(pANCA)and MPO could be found in all cases,positive serum IgG4 was seen in two patients.erythrocyte sedimentation rate(ESR;25-116 mm/1h)and C-reactive protein(CRP;29.02-146.00 mg/L)were both elevated in 14 cases.Nine cases had elevated intracranial pressure[180-235 mmH2O(1 mmH2O=0.0098 kPa)]and abnormal protein level(457.6-3710.0 mg/L)in cerebrospinal fluid.Six cases were treated with glucocorticoids(prednisone 20-60 mg/d)and 9 cased with glucocorticoids and immunosuppressants(methotrexate 15 mg/week or cyclophosphamide 100 mg/d po).All patients achieved remission.MPO-ANCA associated HP is a special type of central nervous system involvement in ANCA associated vasculitis(AAV).It rarely involves the lung or kidney.Steroids and immunosuppressive agents are effective.In HP with unknown underlying diseases,it is suggested to screen ANCA and IgG4 tests for AAV or IgG4-related disease.

关 键 词：肥厚性硬膜炎 髓过氧化物酶 抗中性粒细胞胞质抗体 血管炎 

分 类 号：R742.9[医药卫生—神经病学与精神病学]