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Korean J Med > Volume 86(6); 2014 > Article
The Korean Journal of Medicine 2014;86(6): 749-754.
다기관을 침범한 면역글로불린 G4 비연관성 섬유경화성 대동맥주위염
이지영1, 김지훈1, 이지윤1, 임성희1, 최기홍1, 김정선2, 김덕경1
1성균관대학교 의과대학 삼성서울병원 내과
2성균관대학교 의과대학 삼성서울병원 병리과
Non-IgG4-Related Fibrosclerosing Periaortitis with Multisystemic Involvement
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
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.
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.
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
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