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KMID : 0882420140860060749
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Korean Journal of Medicine
2014 Volume.86 No. 6 p.749 ~ p.754
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Non-IgG4-Related Fibrosclerosing Periaortitis with Multisystemic Involvement
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Lee Ji-Young
Kim Ji-Hoon
Lee Jee-Yun
Lim Sung-Hee
Choi Ki-Hong
Kim Jung-Sun
Kim Duk-Kyung
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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.
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KEYWORD
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Immunoglobulin G, Retroperitoneal fibrosis
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