IgG4-Related Spinal Hypertrophic Pachymeningitis: A Case Report and Systematic Review of 60 Cases

Background: IgG4-related hypertrophic pachymeningitis (IgG4-RHP) is a rare disorder characterized by localized or diffuse fibrotic thickening of the dura mater, affecting both the cranial and spinal regions. IgG4-related spinal hypertrophic pachymeningitis (IgG4-SHP) often manifests with clinical features resembling spinal cord space-occupying lesions, complicating its differential diagnosis. Consequently, establishing an accurate and efficient diagnostic approach is critical for the timely identification and management of IgG4-RHP. Case presentation: We present a patient withIgG4-SHP observed at Zhujiang Hospital of Southern Medical University. The patient's initial symptom was numbness in both lower limbs accompanied by progressive weakness(MRC 2/5 bilaterally). Given the patient's manifestations of spinal cord compression, we initially conducted surgical intervention. The patient was subsequently transferred to the Department of Rheumatology and Immunology, where treatment with steroids was initiated, and then his muscle strength in both lower limbs had improved (MRC 4/5 bilaterally). In accordance with the criteria established by the Japanese Rheumatism Society, IgG4-SHP cases were identified from PubMed, CNKI, and other databases for systematic analysis and literature review (up to November 2024). The data were then analysed via SPSS 26.0 statistical software. Conclusion: We have compiled data from 60 relevant patients for the first time through a literature review. We found that IgG4-SHP predominantly affects middle-aged and elderly patients, with thoracic epidural masses being the most frequently observed manifestation. Clinical presentations, imaging findings, and laboratory investigations often lack specificity, complicating the diagnostic process. A definitive diagnosis relies on the integration of histopathological and immunohistochemical analyses, which provide the most reliable diagnostic criteria. Initial management typically involves corticosteroid therapy. However, in patients presenting with compressive neurological symptoms or suboptimal response to corticosteroids, surgical decompression or adjunctive immunosuppressive agents may be warranted.