Korean J Med.
2015 Jan;88(1):114-119. 10.3904/kjm.2015.88.1.114.
A Case of IgG4-Related Disease with Pachymeningitis and Periaortitis
- Affiliations
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- 1Department of Internal Medicine, Kyungpook National Universitiy School of Medicine, Daegu, Korea. ejnam@knu.ac.kr
- KMID: 2269182
- DOI: http://doi.org/10.3904/kjm.2015.88.1.114
Abstract
- Immunoglobulin G4 (IgG4)-related disease (RD) is an immune-mediated, systemic fibroinflammatory condition characterized by a lymphoplasmacytic infiltration of IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. IgG4-RD has become recognized with increasing frequency since the turn of the century and may affect almost any organ. IgG4-RD also involves the meninges but, to the best of our knowledge, no case of IgG4-related intracranial pachymeningitis with periaortitis has been reported in Korea to date. Here, we report on a 65 year-old male with IgG4-RD involving the meninges and aorta.
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