Clinical features of immunoglobulin-G4-related hypertrophic pachymeningitis
10.3760/cma.j.cn115354-20230310-00137
- VernacularTitle:IgG4相关性肥厚性硬脑膜炎的临床特征分析
- Author:
Huiyao XIANG
1
;
Ming FANG
Author Information
1. 宜昌市第一人民医院神经内科，宜昌 443000
- Keywords:
Immunoglobulin-G4;
Hypertrophic pachymeningitis;
Clinical feature
- From:
Chinese Journal of Neuromedicine
2023;22(7):711-714
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To explore the clinical features of immunoglobulin-G4-related hypertrophic pachymeningitis (IgG4-RHP).Methods:A retrospective analysis on clinical data of a patient with clinically probable IgG4-RHP diagnosed and treated in Yichang First People's Hospital in October 2021 was performed. The clinical data of 45 patients with clinically probable or diagnosed IgG4-RHP publicly reported in journals at home and abroad from October 2012 to October 2022 were retrieved from CNKI, Wanfang Database, and PubMed.Results:Among the 46 patients, 32 were male and 14 were female. The onset age was 55.50, ranged 15-86 years. The most common first symptoms and signs of these patients were headache (39.1%, 18/46) and visual impairment (32.6%, 15/46). All 46 patients showed meningeal enhancement on cranial MRI (plain scan+enhanced scan), usually involving in one cerebral hemisphere (37.0%, 17/46). 80% patients (32/40) had elevated serum IgG4 level, 60% patients (12/20) were combined with anti-neutrophil cytoplasmic antibody positive changes, and 73.9% patients (17/23) had abnormal changes in cerebrospinal fluid. All 30 patients who completed the brain tissue pathological biopsy showed IgG4 + plasma cell infiltration of lymph and plasma cells, accompanied by mat fibrosis, obliterated phlebitis and eosinophilic infiltration; IgG4 cells accounted for more than 40% of IgG cells, and IgG4 cells were more than 10 in each high-power view field. Among 46 patients, 1 patient died, 1 remained disabled and 6 relapsed; the prognosis of the remaining patients was good. Conclusion:IgG4-RHP mostly occurs in middle-aged subjects, without gender difference or specific clinical manifestations; headache is the most common initial symptom, unilateral hemisphere can be involved, and elevated serum IgG4 can be accompanied.
