MPO-ANCA相关性肥厚性硬脑膜炎合并肉芽肿性血管炎一例

A Case of Hypertrophic Pachymeningitis Associated with MPO-ANCA and Granulomatous Vasculitis

摘要: 目的：本研究报道一例MPO-ANCA相关性肥厚性硬脑膜炎合并肉芽肿性血管炎导致剧烈头痛的临床病例，旨在为该疾病的诊断和治疗提供临床参考依据。方法：通过对该病例的系统分析，并结合文献复习，探讨其临床特征及治疗方法。结果：病例分析显示，该患者为中年男性，病史复杂，主要表现为剧烈头痛，其病因难以确定，经手术活检及辅助检查后确诊并通过免疫抑制及激素治疗后头痛缓解出院。结论：本案例提示：肥厚性硬脑膜炎是以颅脑或脊髓硬膜慢性、进行性的炎症肥厚性改变为病理表现的罕见病，其中，抗中性粒细胞胞质抗体(ANCA)相关性血管炎(AAV)是一种自身免疫性疾病，可累及全身多个系统，如未进行治疗，其平均存活时间仅为半年。此疾病应早期识别并治疗，避免误诊漏诊。

Abstract: Objective: This study reports a clinical case of MPO-ANCA associated hypertrophic pachymeningitis with granulomatous vasculitis causing severe headache, aiming to provide clinical reference for the diagnosis and treatment of this disease. Methods: Through systematic analysis of the case and literature review, the clinical features and treatment methods were explored. Results: The case analysis showed that the patient was a middle-aged male with a complex medical history, mainly presenting with severe headache. The cause was difficult to determine. After surgical biopsy and auxiliary examinations, the diagnosis was confirmed and the headache was relieved after immunosuppressive and hormone therapy, and the patient was discharged. Conclusion: This case suggests that hypertrophic pachymeningitis is a rare disease characterized by chronic and progressive inflammatory thickening of the dura mater of the brain or spinal cord. Among them, antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is an autoimmune disease that can affect multiple systems throughout the body. If left untreated, the average survival time is only half a year. This disease should be identified and treated early to avoid misdiagnosis and missed diagnosis.