Non-IgG4-Related Fibrosclerosing Periaortitis with Multisystemic Involvement.
- Author:
Ji Young LEE
1
;
Ji Hoon KIM
;
Ji Yun LEE
;
Sung Hee LIM
;
Ki Hong CHOI
;
Jung Sun KIM
;
Duk Kyung KIM
Author Information
1. Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. dukkyung.kim@samsung.com
- Publication Type:Case Report
- Keywords:
Immunoglobulin G;
Retroperitoneal fibrosis
- MeSH:
Adventitia;
Aged;
Aorta;
Ascites;
Female;
Humans;
Immunoglobulin G;
Pancytopenia;
Pleural Effusion;
Retroperitoneal Fibrosis
- From:Korean Journal of Medicine
2014;86(6):749-754
- CountryRepublic of Korea
- Language:Korean
-
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.
