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http://dx.doi.org/10.3904/kjm.2014.86.6.749

Non-IgG4-Related Fibrosclerosing Periaortitis with Multisystemic Involvement  

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)
Publication Information
The Korean Journal of Medicine / v.86, no.6, 2014 , pp. 749-754 More about this Journal
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
Immunoglobulin G; Retroperitoneal fibrosis;
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