다기관을 침범한 면역글로불린 G4 비연관성 섬유경화성 대동맥주위염
Non-IgG4-Related Fibrosclerosing Periaortitis with Multisystemic Involvement
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초록
Fibrosclerosing periaortitis is a rare condition that refers to a spectrum of idiopathic diseases characterized by a fibroinflammatoryreaction that extends from the adventitia of the aorta into the surrounding structures. It can be present in eitherIgG4-related or non-IgG4-related fibrosclerosing periaortitis. IgG4-related fibrosclerosing periaortitis could be associated withconcomitant multi-organ involvement. However, non-IgG4-related fibrosclerosing periaortitis has rarely been associated withsystemic manifestations. Here, we report a 76-year-old female with non-IgG4-related fibrosclerosing periaortitis, who developedpancytopenia, pleural effusion and ascites, which improved after high dose steroid treatment.
Abstract
Fibrosclerosing periaortitis is a rare condition that refers to a spectrum of idiopathic diseases characterized by a fibroinflammatoryreaction that extends from the adventitia of the aorta into the surrounding structures. It can be present in eitherIgG4-related or non-IgG4-related fibrosclerosing periaortitis. IgG4-related fibrosclerosing periaortitis could be associated withconcomitant multi-organ involvement. However, non-IgG4-related fibrosclerosing periaortitis has rarely been associated withsystemic manifestations. Here, we report a 76-year-old female with non-IgG4-related fibrosclerosing periaortitis, who developedpancytopenia, pleural effusion and ascites, which improved after high dose steroid treatment.
참고문헌 (10)
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