ANCA相关血管炎并发肥厚性硬脊髓膜炎6例临床特点

Clinical features of six cases of ANCA-associated vasculitis with hypertrophic spinal pachymeningitis

目的探讨ANCA相关血管炎并发肥厚性硬脊髓膜炎的临床与影像学特点、治疗方法及疾病转归。方法回顾性分析2014年1月至2020年1月北京协和医院风湿免疫科收治的6例ANCA相关血管炎(ANCA-associated vasculitis,AAV)并发肥厚性硬脊髓膜炎患者的临床资料,并进一步总结其治疗及预后。结果6例患者,均为女性。AAV起病年龄中位数为50.5(26~66)岁。其中1例为肉芽肿性多血管炎,5例为未分类AAV。临床主要表现有发热、乏力、颈肩腰背痛、躯干及肢体麻木。6例患者的血沉平均值为(79.17±39.3)mm/h,C反应蛋白中位数为77.53(36.45~169.45)mg/L。6例患者脑脊液蛋白中位数为13.95(1.36~36.08)g/L,均存在异常淋巴细胞激活。增强核磁共振扫描显示硬脊髓膜增厚,在T1WI主要呈低或等信号,在T2WI主要呈低或等信号,增强后明显强化。6例患者均给予激素冲击或大剂量糖皮质激素联合环磷酰胺治疗,5例给予鞘内注射(地塞米松或地塞米松联合甲氨蝶呤)治疗。平均随访60个月,6例患者症状均改善,5例患者影像学缓解。结论肥厚性硬脊髓膜炎是AAV罕见的临床表现,脊髓增强核磁和脑脊液检查有助于诊断。糖皮质激素联合环磷酰胺是治疗的首选,可考虑联合鞘内注射治疗。

Objective To analyze the clinical features,spinal image,treatment,and prognosis of ANCA-associated vasculitis(AAV)with hypertrophic spinal pachymeningitis(HSP).Methods We retrospectively analyzed the clinical manifestations,laboratory examinations,spinal contrast-enhanced MRI image,treatment,and prognosis of 6 patients of AAV with HSP in Peking Union Medical College Hospital from 2014 to 2020.Results All of the 6 patients were female.The median age at onset of AAV was 50.5(26-66)years old.Among the 6 patients,1 patient was diagnosed with granulomatosis with polyangiitis and 5 patients were diagnosed with unclassified AAV.The major clinical symptoms were fever,fatigue,neck shoulder pain and back pain,numbness of trunk and limbs.The mean level of ESR was(79.17±39.3)mm/h,and the median CRP was 77.53(36.45-169.45)mg/L.The median cerebrospinal fluid(CSF)protein was 13.95(1.36-36.08)g/L.CSF cytological analysis suggested abnormal lymphocyte activation.The contrast-enhanced MRI scan of spinal cord demonstrated diffuse spinal dura matter thickening.Six patients received steroid pulse or high dose glucocorticoid therapy combined with cyclophosphamide treatment,5 patients received intrathecal injection of dexamethasone with or without methotrexate.The average follow-up period was 60 months,symptoms of all 6 patients were relieved and remained stable,5 patients achieved imaging remission.Conclusions HSP is a rare clinical manifestation of AAV.Spinal contrast-enhanced MRI and CSF examination are helpful for diagnosis.Combination therapy of corticosteroids and cyclophosphamide is the first-line choice of treatment,concurrent intrathecal injection can be considered.