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Non-IgG4-Related Fibrosclerosing Periaortitis with Multisystemic Involvement / 대한내과학회지
Korean Journal of Medicine ; : 749-754, 2014.
Article in Korean | WPRIM | ID: wpr-85489
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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Full text: Available Index: WPRIM (Western Pacific) Main subject: Aorta / Pancytopenia / Pleural Effusion / Ascites / Retroperitoneal Fibrosis / Immunoglobulin G / Adventitia Limits: Aged / Female / Humans Language: Korean Journal: Korean Journal of Medicine Year: 2014 Type: Article

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Full text: Available Index: WPRIM (Western Pacific) Main subject: Aorta / Pancytopenia / Pleural Effusion / Ascites / Retroperitoneal Fibrosis / Immunoglobulin G / Adventitia Limits: Aged / Female / Humans Language: Korean Journal: Korean Journal of Medicine Year: 2014 Type: Article