A Case of IgG4-Related Disease with Pachymeningitis and Periaortitis / 대한내과학회지

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.