A Case of IgG4 Related Pachymeningitis

Abstract

Background Immunoglobulin G4 (IgG4) related disease is an immune-mediated fibroinflammatory condition that can affect multiple organs. There are only few reports of pachymeningeal involvement by IgG4 related disease in Korea. Case Report A 50-year-old woman was admitted due to generalized seizure and headache. Brain magnetic resonance imaging (MRI) showed nodular dural thickening and enhancement in the right frontal areas. A meningeal biopsy was performed. On pathology, chronic inflammation, fibrosis and plasma cell infiltration were revealed. Immunoglobulin G4 immunohistochemistry showed prominent IgG4+ plasma cell infiltrate. Serum IgG level was normal but IgG4 subclass level was significantly elevated. She was treated with intravenous dexamethasone followed by oral prednisolone. After 1 year of treatment, brain MRI showed marked decrease of dural thickening and enhancement. Conclusion We report a case of IgG4 related pachymeningitis improved with corticosteroid. IgG4 related disease should be included in the differential diagnosis in patients with hypertrophic pachymeningitis.

Keywords

• Immunoglobulin G4
• Pachymeningitis
• Corticosteroid