비후성 뇌수막염과 대동맥주위염으로 발현한 IgG4 연관 질환 1예
A Case of IgG4-Related Disease with Pachymeningitis and Periaortitis
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초록
IgG4 연관 질환은 모든 신체 장기를 침범할 수 있는 자가면역성 염증 질환으로 진단에 있어 침범 장기의 조직학적소견이 가장 중요하다. IgG4 연관 질환이 뇌수막염, 대동맥주위염 등의 다양한 형태로 발현되지만, 뇌수막염과 대동맥주위염이 동시에 발현된 경우는 세계적으로 매우 드물며 국내에 보고된 바가 없다. 이에 저자들은 비후성 뇌수막염과대동맥주위염을 동반한 환자에서 조직학적으로 증명된 IgG4연관 질환 증례 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.
Abstract
Immunoglobulin G4 (IgG4)-related disease (RD) is an immune-mediated, systemic fibroinflammatory condition characterizedby a lymphoplasmacytic infiltration of IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. IgG4-RD has becomerecognized with increasing frequency since the turn of the century and may affect almost any organ. IgG4-RD also involvesthe meninges but, to the best of our knowledge, no case of IgG4-related intracranial pachymeningitis with periaortitis has been reportedin Korea to date. Here, we report on a 65 year-old male with IgG4-RD involving the meninges and aorta.
참고문헌 (10)
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