摘要： 目的 报道2例IgG4相关硬脊膜炎（IgG4-RSP）患者并复习相关文献，总结IgG4-RSP的临床特征。方法 与结果 首都医科大学宣武医院2023年诊断与治疗2例IgG4-RSP患者，均为女性，55和63岁，临床主要表现为脊髓压迫症状；脊柱MRI显示硬脊膜增厚，增强扫描呈明显强化征象；血清IgG4正常，脑脊液白细胞计数增加、蛋白定量升高；硬脊膜组织活检可见大量淋巴细胞、浆细胞浸润，硬脊膜纤维化，IgG4⁺细胞数目增加。最终诊断为IgG4-RSP，予手术减压、糖皮质激素和免疫抑制剂治疗后，症状明显改善。结论 IgG4-RSP以中年患者多见，无性别差异，主要累及颈胸髓，多以脊髓压迫致疼痛和肢体麻木无力为首发症状，近40%患者血清IgG4正常，故明确诊断依靠硬脊膜组织活检术，手术减压、糖皮质激素和免疫抑制剂治疗有效。

关键词: 免疫球蛋白G4相关疾病, 脊髓疾病, 脑脊髓液, 磁共振成像, 病理学

Abstract: Objective Two cases of IgG4 -related hypertrophic spinal pachymeningitis (IgG4 -RSP) were reported, and literatures were reviewed, in order to summarize the clinical characteristics of IgG4-RSP. Methods and Results The 2 female patients were diagnosed and treated at Xuanwu Hospital, Capital Medical University in 2023, aged 55 and 63 years old, respectively. The primary manifestations was spinal cord compression. Spinal cord MRI showed spinal dura mater thickening with enhancement. Serum IgG4 levels are within the normal range, while cerebrospinal fluid analysis showed an increased white blood cell count and elevated protein levels. Dural biopsy findings indicate extensive lymphocytes and plasma cells infiltration, spinal dura mater fibrosis, and a significant increase in IgG4⁺ cell counts. Based on these findings, the final diagnosis was IgG4-RSP. Following surgical decompression, along with glucocorticoid and immunosuppressive therapy, there was a marked improvement in symptoms. Conclusions IgG4-RSP is more common in middle-aged patients, with no gender difference. It mainly affects the cervical and thoracic spinal cord. The initial symptoms are often pain and numbness and weakness in the limbs due to spinal cord compression. Nearly 40% of patients have normal serum IgG4 levels. Therefore, the definite diagnosis mainly relies on the biopsy of the spinal dura mater. Surgical decompression, glucocorticoids and immunosuppressants are effective treatments.

Key words: Immunoglobulin G4-related disease, Spinal cord diseases, Cerebrospinal fluid, Magnetic resonance imaging, Pathology