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Case Report
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Korean J Med. 2014;86(6):749-754. Published online June 1, 2014.
DOI: https://doi.org/10.3904/kjm.2014.86.6.749
- 다기관을 침범한 면역글로불린 G4 비연관성 섬유경화성 대동맥주위염
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이지영1, 김지훈1, 이지윤1, 임성희1, 최기홍1, 김정선2, 김덕경1
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1성균관대학교 의과대학 삼성서울병원 내과
2성균관대학교 의과대학 삼성서울병원 병리과
- Non-IgG4-Related Fibrosclerosing Periaortitis with Multisystemic Involvement
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Ji Young Lee1, Ji Hoon Kim1, Ji Yun Lee1, Sung Hee Lim1, Ki Hong Choi1, Jung-Sun Kim2, Duk-Kyung Kim1
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1Departments of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
2Departments of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
- Corresponding author: Duk-Kyung Kim ,Tel: +82-2-3410-3419, Fax: +82-2-3410-3849, Email: dukkyung.kim@samsung.com
- Received: June 19, 2013; Revised: July 26, 2013 Accepted: August 6, 2013.
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߽ɾ :면역글로불린 G4 비연관성 대동맥주위염; 후복막 섬유증
- 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. (Korean J Med 2014;86:749-754)
Keywords :Immunoglobulin G, Retroperitoneal fibrosis
