| 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 |
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 |
| 다기관을 침범한 면역글로불린 G4 비연관성 섬유경화성 대동맥주위염 |
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이지영1, 김지훈1, 이지윤1, 임성희1, 최기홍1, 김정선2, 김덕경1 |
1성균관대학교 의과대학 삼성서울병원 내과
2성균관대학교 의과대학 삼성서울병원 병리과 |
Correspondence:
Duk-Kyung Kim, Tel: +82-2-3410-3419, Fax: +82-2-3410-3849, Email: dukkyung.kim@samsung.com
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Received: 19 June 2013 • Revised: 26 July 2013 • Accepted: 6 August 2013 |
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| This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
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| Abstract |
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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) |
| Key Words:
Immunoglobulin G; Retroperitoneal fibrosis |
| 주제어:
면역글로불린 G4 비연관성 대동맥주위염; 후복막 섬유증 |
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