IgG4相关性肥厚性硬脑膜炎的临床特征分析

Clinical features of immunoglobulin-G4-related hypertrophic pachymeningitis

目的探讨IgG4相关性肥厚性硬脑膜炎(IgG4-RHP)的临床特征。方法回顾性报道2021年10月宜昌市第一人民医院神经内科诊治的1例临床很可能IgG4-RHP患者的病历资料,并检索中国知网、万方、PubMed等数据库,筛选自2012年10月至2022年10月收录的IgG4-RHP相关研究,收集45例临床很可能或确诊IgG4-RHP患者的个案资料,汇总分析IgG4-RHP的临床特征。结果46例患者中,男性32例,女性14例;发病年龄为55.50岁,范围为15~86岁。患者最常见的首发症状及主要症状体征分别为头痛(39.1%,18/46)和视力障碍(32.6%,15/46)。头颅MRI平扫+增强扫描均示患者硬脑膜局灶性或弥漫性增厚,一侧大脑半球受累最常见(37.0%,17/46)。80%(32/40)患者的血清IgG4水平升高,60%(12/20)患者合并抗中性粒细胞胞浆抗体阳性改变,73.9%(17/23)患者的脑脊液有异常改变。完成脑组织病理活检的30例患者均提示有以IgG4+浆细胞为主的淋巴细胞、浆细胞浸润,并伴有席纹状纤维化、闭塞性静脉炎和嗜酸性粒细胞浸润;IgG4细胞占IgG细胞比值>40%,且每高倍镜视野中IgG4细胞>10个。46例患者中死亡1例,遗留残疾1例,症状复发6例,余患者均预后良好。结论IgG4-RHP多发生于中年人,无性别差异,临床表现缺乏特异性,头痛是最常见的首发症状,易受累区域通常为一侧大脑半球,多数患者伴有血清IgG4水平的升高。

Objective To explore the clinical features of immunoglobulin-G4-related hypertrophic pachymeningitis(IgG4-RHP).MethodsA retrospective analysis on clinical data of a patient with clinically probable IgG4-RHP diagnosed and treated in Yichang First People's Hospital in October 2021 was performed.The clinical data of 45 patients with clinically probable or diagnosed IgG4-RHP publicly reported in journals at home and abroad from October 2012 to October 2022 were retrieved from CNKI,Wanfang Database,and PubMed.ResultsAmong the 46 patients,32 were male and 14 were female.The onset age was 55.50,ranged 15-86 years.The most common first symptoms and signs of these patients were headache(39.1%,18/46)and visual impairment(32.6%,15/46).All 46 patients showed meningeal enhancement on cranial MRI(plain scan+enhanced scan),usually involving in one cerebral hemisphere(37.0%,17/46).80%patients(32/40)had elevated serum IgG4 level,60%patients(12/20)were combined with anti-neutrophil cytoplasmic antibody positive changes,and 73.9%patients(17/23)had abnormal changes in cerebrospinal fluid.All 30 patients who completed the brain tissue pathological biopsy showed IgG4+plasma cell infiltration of lymph and plasma cells,accompanied by mat fibrosis,obliterated phlebitis and eosinophilic infiltration;IgG4 cells accounted for more than 40%of IgG cells,and IgG4 cells were more than 10 in each high-power view field.Among 46 patients,1 patient died,1 remained disabled and 6 relapsed;the prognosis of the remaining patients was good.ConclusionIgG4-RHP mostly occurs in middle-aged subjects,without gender difference or specific clinical manifestations;headache is the most common initial symptom,unilateral hemisphere can be involved,and elevated serum IgG4 can be accompanied.