Korean J Med.
2014 Jun;86(6):749-754.
Non-IgG4-Related Fibrosclerosing Periaortitis with Multisystemic Involvement
- Affiliations
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- 1Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. dukkyung.kim@samsung.com
- 2Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
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.
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