抗中性粒细胞胞浆抗体相关性肥厚性硬脊膜炎1例报告并文献复习

Anti-neutrophil cytoplasmic antibody associated hypertrophic spinal pachymeningitis: A case report and literature review

目的探讨抗中性粒细胞胞浆抗体(ANCA)相关性肥厚性硬脊膜炎(HSP)的临床特点、MRI表现。方法报道1例ANCA相关性HSP患者的临床资料,搜索国内外报道的ANCA相关性HSP患者14例,统计分析该类患者的流行病学特点、临床表现、MRI特征、治疗及预后情况。结果该患者以进行性加重的腰痛,双下肢麻木、无力起病,检查见血沉增快,C反应蛋白升高、p-ANCA(+),胸椎MRI增强见硬脊膜明显增厚伴强化,背侧明显,脊髓明显受压变性。予以激素治疗症状明显好转。统计分析显示ANCA相关性HSP女性多见,中老年发病,慢性病程,进行性加重。以局部疼痛、神经根和脊髓压迫症状为主要表现,血炎症指标升高,以p-ANCA(+)为多见。胸段受累最多,受累节段较长,病灶强化明显,多呈条索状,背侧增厚明显。激素和免疫抑制剂治疗有效,多数预后良好。结论 ANCA相关性HSP主要以进行性加重的颈背腰部疼痛、节段以下肢体无力和感觉异常为主要表现,血液炎性指标检测、MRI增强检查有助于诊断,激素和免疫抑制剂治疗有效,多数患者预后良好。

Objective To investigate the clinical features and MRI findings of anti-neutrophil cytoplasmic antibody( ANCA) associated hypertrophic spinal meningitis( HSP). Methods A case of ANCA-related HSP was reported. 14 cases of ANCA-related HSP reported were searched. The epidemiological characteristics,clinical manifestations,MRI features,treatment and prognosis of these patients were analyzed. Result With progressive aggravation of low back pain,numbness and inability to start the disease,the examination showed that ESR increased,C-reactive protein increased,p-ANCA(+).thoracic spinal MRI enhanced showed marked thickening and enhancement of dura mater,obvious dorsal side,and marked compression degeneration of spinal cord. The symptoms improved significantly after hormone treatment. Statistical analysis showed that ANCA-associated HSP was more common in females,middle-aged and elderly patients,chronic course of disease,progressive aggravation. Local pain,nerve root and spinal cord compression symptoms were the main manifestations.Blood inflammation index was increased,and p-ANCA(+) was the most common manifestation. The thoracic segment was most affected,the involved segment was longer,the lesion was obviously enhanced,mostly in the shape of a cord,and the dorsal thickening was obvious. Hormones and immunosuppressants are effective and most patients with them have good prognosis. Conclusion ANCA-associated HSP is mainly manifested by progressive neck and back pain,lower limb weakness and sensory abnormalities. Detection of blood inflammatory markers and enhanced MRI are helpful for diagnosis. Hormones and immunosuppressive agents are effective in the treatment of HSP. Most patients have good prognosis.