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KMID : 0882420150880010114
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Korean Journal of Medicine
2015 Volume.88 No. 1 p.114 ~ p.119
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A Case of IgG4-Related Disease with Pachymeningitis and Periaortitis
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Eun Jung-Su
Kwon Sang-Hoon
Lee Eun-Song
Kang Young-Mo
Nam Eon-Jeong
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Abstract
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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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KEYWORD
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Immunoglobulin G4, Meningitis, Periaortitis
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