IgG4-related dural meningitis: two cases report and literature review

doi:10.3969/j.issn.1672-6731.2025.12.010

Abstract

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

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。

Hui LU, Bing-xue ZHANG. IgG4-related dural meningitis: two cases report and literature review[J]. Chinese Journal of Contemporary Neurology and Neurosurgery, 2025, 25(12): 1163-1172.

陆慧, 张冰雪. IgG4相关硬脊膜炎两例并文献复习[J]. 中国现代神经疾病杂志, 2025, 25(12): 1163-1172.

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URL: https://journal11.magtechjournal.com/cjcnn/EN/10.3969/j.issn.1672-6731.2025.12.010

https://journal11.magtechjournal.com/cjcnn/EN/Y2025/V25/I12/1163

Figures/Tables 4

Figure 1 Spinal cord MRI findings Sagittal T₂WI showed C₅-T₂ spinal cord hypointensity ventrally and dorsally (arrows indicate), considering thickened spinal dura mater and adjacement vertebral canal stenosis (Panel 1a). Sagittal fat suppression T₁WI showedthickened spinal dura mater enhancement (arrow indicates, Panel 1b). Axial T₂WI showed dorsal compression of the spinal cord C₇-T₁(arrow indicates, Panel 1c). Axial fat suppression enhanced T₁WI showed uneven enhancement of the ventral spinal dura mater (arrowindicates, Panel 1d). Sagittal T₂WI showed that the ventral fusiform hypointensity of the L_1-3 spinal cord (arrow indicates), and theventral compression of the spinal cord (Panel 1e). Sagittal fat suppression enhanced T₁WI showed there was no obvious enhancement inthe center of the lesion, and the posterior margin of the vertebral was strengthened (arrow indicates, Panel 1f). Axial T₂WI showedfusiform hypointensity on the ventral aspects within the L_2-3 vertebral canal (arrow indicates, Panel 1g). Axial fat suppression enhancedT₁WI showed no significant enhancement in the center of lesion (arrow indicates, Panel 1h).

Figure 2 Light microscopy findings Storiform fibrosis (Panel 2a). HE staining Low power magnified A large number oflymphocytes, plasma cells infiltration (Panel 2b). HE staining High power magnified IgG was positive (Panel 2c).Immunohistochemical staining (EnVision) Medium power magnified Local IgG4⁺ cells were 15 /HPF (Panel 2d).Immunohistochemical staining (EnVision) Medium power magnified

Figure 3 Head and cervical vertebra MRI findings Sagittal T₂WI showed hypointensity at the posterior margin of thecervical and thoracic vertebrae (arrow indicates, Panel 3a). Sagittal enhanced T₁WI showed thickening of the spinal dura materat the posterior margin of the cervical and thoracic vertebrae with significant enhancement (arrow indicates, Panel 3b). Sagittalenhanced T₁WI showed significant enhancement of the spinal dura mater in the cerebellum, medulla oblongata and uppercervical segment (arrows indicate, Panel 3c). Axial T₂WI showed compression of the anterior margin of the thoracic vertebralcanal and ventral surface of the thoracic spinal cord (arrow indicates, Panel 3d). Axial enhanced T₁WI showed thickening ofthe spinal dura mater in the upper cervical segment, with significant enhancement (arrow indicates), and evident compressionand flattening of the adjacent spinal cord (Panel 3e). Axial enhanced T₁WI showed thickening of the bilateral posterior cranialfossa cerebral dura mater accompanied by abnormal enhancement (arrow indicates, Panel 3f).

Figure 4 Light microscopy findings Storiform fibrosis (Panel 4a). HE staining Low power magnified A large number oflymphocyte, plasma cell infiltration (Panel 4b). HE staining High power magnified IgG was positive (Panel 4c).Immunohistochemical staining (EnVision) Medium power magnified Local IgG4⁺ cells were 40/HPF (Panel 4d).Immunohistochemical staining (EnVision) Medium power magnified

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