抗中性粒细胞胞质抗体相关性肥厚性硬脊膜炎一例及文献综述  

ANCA-associated hypertrophic spinal pachymeningitis:a case report and literature review

作　　者：陈盛博 文柱 谢远龙[3] 邓洲铭[3] CHEN Sheng-bo;WEN Zhu;XIE Yuan-long;DENG Zhou-ming(Department of Orthopedics,People's Hospital of Qichun County,Huanggang,Hubei,435300,China)

机构地区：[1]蕲春县人民医院骨科,黄冈435300 [2]罗田县万密斋医院骨科,黄冈438600 [3]武汉大学中南医院脊柱与骨肿瘤外科,湖北省430061

出　　处：《中国骨与关节杂志》2025年第4期325-330,共6页Chinese Journal of Bone and Joint

摘　　要：目的报道经我院病理证实的1例肥厚性硬脊膜炎(hypertrophic spinal pachymeningitis,HSP),结合文献报道17例(总计18例),探讨HSP的临床疾病特征。方法介绍并分析本院经治的1例由HSP患者住院期间突发下肢肌力下降术后肌力恢复的临床病例特点。检索中英文数据库,包括发病部位、影像特征、相关抗体特征、病理特征、治疗方法及预后,对检索文献进行分析。结果统计的18例HSP患者中,女性(11例)多于男性(7例),与抗中性粒细胞胞质抗体(antineutrophil cytoplasmic antibodies,ANCA)相关居首位(占比39%),颈胸段、胸段发病率最高(83%),治疗上以激素治疗为主,部分需要手术。结论HSP是一种以硬脊膜增厚和纤维化为主要特征的少见疾病,早期诊断和个体化治疗对于改善患者预后至关重要。然而,由于其罕见性,临床医师需提高对该疾病的认识,结合临床表现、影像学、实验室检查及病理结果综合判断,以制订精准的治疗方案。Objective To report 1 case of hypertrophic spinal pachymeningitis confirmed by the pathology department of our hospital and review 17 cases,with a total of 18 cases.To explore the clinical characteristics of hypertrophic spinal pachymeningitis.Methods Introduce and analyze the clinical case characteristics of a patient with hypertrophic spinal pachymeningitis who experienced a sudden decrease in lower limb muscle strength during hospitalization and recovered muscle strength after surgery in our hospital.Search Chinese and English databases,including the location of onset,imaging features,characteristics of related antibodies,pathological features,treatment methods,and prognosis,and analyze the retrieved literature.Results Among the 18 HSP cases in the statistics,there were more females(11 cases)than males(7 cases).The ANCA-associated cases ranked first,accounting for 39%.The incidence was the highest in the cervicothoracic and thoracic segments,accounting for 83%.Hormone therapy was the main treatment,and some cases required surgery.Conclusions Hypertrophic spinal pachymeningitis is a rare disease mainly characterized by thickening and fibrosis of the spinal dura mater.It can coexist with hypertrophic cranical pachymeningitis(HCP).Early diagnosis and individualized treatment are crucial for improving the prognosis of patients.However,due to its rarity,clinicians need to enhance their understanding of this disease and make a comprehensive judgment by combining clinical manifestations,imaging,laboratory tests,and pathological results to formulate an accurate treatment plan.

关 键 词：脊柱 抗体 抗中性白细胞胞质 肥厚性硬脊膜炎 

分 类 号：R687.3[医药卫生—骨科学]