다기관을 침범한 면역글로불린 G4 비연관성 섬유경화성대동맥주위염

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.