IgG4-related dural meningitis:two cases report and literature review
LU Hui
ZHANG Bing-xue
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.
Keywords：Immunoglobulin G4-related diseaseSpinal cord diseasesCerebrospinal fluidMagnetic resonance imagingPathology
Publication Date：2025-12-25
Online Publishing Date：2026-01-23(First online date of this platform, not the publication date of the document)
Pages：10( 1163-1172 )