IgG4-related hypertrophic pachymeningitis： A clinical analysis of seven cases
10.19845/j.cnki.zfysjjbzz.2025.0204
- VernacularTitle:IgG4相关性肥厚性硬脑膜炎7例临床分析
- Author:
Xue WANG
1
;
Hongyan BI
1
Author Information
1. Department of Neurology， Capital Medical University Affiliated Beijing Friendship Hospital， Beijing 100050， China
- Publication Type:Journal Article
- Keywords:
Immunoglobulin G4-related disease;
Hypertrophic pachymeningitis;
Diagnosis;
Treatment
- MeSH:
Diagnosis
- From:
Journal of Apoplexy and Nervous Diseases
2025;42(12):1131-1135
- CountryChina
- Language:Chinese
-
Abstract:
Objective Immunoglobulin G4-related hypertrophic pachymeningitis （IgG4-RHP） is a rare autoimmune disorder. This study aims to conduct a clinical analysis of seven cases with IgG4-RHP to enhance the understanding of this disease. Methods A retrospective analysis was conducted on seven patients diagnosed with IgG4-RHP. The demographic data， clinical manifestations， laboratory examinations， cerebrospinal fluid analysis， imaging， pathological manifestations， treatments， and efficacy analysis data were collected. Results The patients’ ages ranged from 38 to 67 years， with most being males. Headache （6/7） and cranial nerve palsies （5/7） were the most common clinical symptoms. Notably， one case presented with seizures and another with limb weakness. All patients exhibited elevated serum IgG4 levels （1.48-3.25 g/L）. Imaging studies revealed varying degrees of dural thickening and enhancement located in the global dura， tentorium cerebelli， cavernous sinus， and thoracic spinal dura. A definitive pathological diagnosis was obtained via biopsy （intracranial， nasal sinus， or orbital） in 3 patients. All patients received glucocorticoid therapy， with 5 receiving combined cyclophosphamide due to extensive or severe lesions. At the 6-month follow-up， 2 patients achieved complete response， 4 showed partial response， and 1 showed no response. During long-term follow-up， one patient experienced relapse during steroid tapering， which ended up in response again after immunosuppressive regimen adjustment. Conclusion IgG4-RHP is characterized by significant heterogeneous manifestations， which may involve the spinal cord or induce seizures. Diagnosis requires a combination of serological， radiological， and pathological results. While glucocorticoids with immunosuppressants are the main treatment measures， patients with long disease duration and significant fibrosis may respond poorly， highlighting the importance of early intervention and the exploration of novel therapies for refractory cases.
- Full text:2026012308290430667IgG4相关性肥厚性硬脑膜炎7例临床分析.pdf
