The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
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Non-IgG4-Related Fibrosclerosing Periaortitis with Multisystemic Involvement

다기관을 침범한 면역글로불린 G4 비연관성 섬유경화성 대동맥주위염

  • Lee, Ji Young (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kim, Ji Hoon (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lee, Ji Yun (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lim, Sung Hee (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Choi, Ki Hong (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kim, Jung-Sun (Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kim, Duk-Kyung (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • 이지영 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 김지훈 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 이지윤 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 임성희 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 최기홍 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 김정선 (성균관대학교 의과대학 삼성서울병원 병리과) ;
  • 김덕경 (성균관대학교 의과대학 삼성서울병원 내과)
  • Received : 2013.06.19
  • Accepted : 2013.08.06
  • Published : 2014.06.01
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Abstract

Fibrosclerosing periaortitis is a rare condition that refers to a spectrum of idiopathic diseases characterized by a fibroinflammatory reaction that extends from the adventitia of the aorta into the surrounding structures. It can be present in either IgG4-related or non-IgG4-related fibrosclerosing periaortitis. IgG4-related fibrosclerosing periaortitis could be associated with concomitant multi-organ involvement. However, non-IgG4-related fibrosclerosing periaortitis has rarely been associated with systemic manifestations. Here, we report a 76-year-old female with non-IgG4-related fibrosclerosing periaortitis, who developed pancytopenia, pleural effusion and ascites, which improved after high dose steroid treatment.

Keywords

References

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