A Case of IgG4-Related Disease with Pachymeningitis and Periaortitis.
10.3904/kjm.2015.88.1.114
- Author:
Jung Su EUN
1
;
Sang Hoon KWON
;
Eun Song LEE
;
Young Mo KANG
;
Eon Jeong NAM
Author Information
1. Department of Internal Medicine, Kyungpook National Universitiy School of Medicine, Daegu, Korea. ejnam@knu.ac.kr
- Publication Type:Case Report
- Keywords:
Immunoglobulin G4;
Meningitis;
Periaortitis
- MeSH:
Aorta;
Fibrosis;
Humans;
Immunoglobulins;
Korea;
Male;
Meninges;
Meningitis*;
Phlebitis;
Plasma Cells
- From:Korean Journal of Medicine
2015;88(1):114-119
- CountryRepublic of Korea
- Language:Korean
-
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.
