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다기관을 침범한 면역글로불린 G4 비연관성 섬유경화성 대동맥주위염

Non-IgG4-Related Fibrosclerosing Periaortitis with Multisystemic Involvement

  • 이지영 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 김지훈 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 이지윤 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 임성희 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 최기홍 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 김정선 (성균관대학교 의과대학 삼성서울병원 병리과) ;
  • 김덕경 (성균관대학교 의과대학 삼성서울병원 내과)
  • Lee, Ji Young (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kim, Ji Hoon (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lee, Ji Yun (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lim, Sung Hee (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Choi, Ki Hong (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kim, Jung-Sun (Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kim, Duk-Kyung (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • 투고 : 2013.06.19
  • 심사 : 2013.08.06
  • 발행 : 2014.06.01

초록

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.

키워드

참고문헌

  1. Vaglio A, Greco P, Corradi D, et al. Autoimmune aspects of chronic periaortitis. Autoimmun Rev 2006;5:458-464. https://doi.org/10.1016/j.autrev.2006.03.011
  2. Vaglio A, Buzio C. Chronic periaortitis: a spectrum of diseases. Curr Opin Rheumatol 2005;17:34-40. https://doi.org/10.1097/01.bor.0000145517.83972.40
  3. Sakamoto A, Nagai R, Saito K, et al. Idiopathic retroperitoneal fibrosis, inflammatory aortic aneurysm, and inflammatory pericarditis: retrospective analysis of 11 case histories. J Cardiol 2012;59:139-146. https://doi.org/10.1016/j.jjcc.2011.07.014
  4. Kasashima S, Zen Y, Kawashima A, Endo M, Matsumoto Y, Kasashima F. A new clinicopathological entity of IgG4-related inflammatory abdominal aortic aneurysm. J Vasc Surg 2009;49:1264-1271. https://doi.org/10.1016/j.jvs.2008.11.072
  5. Kasashima S, Zen Y, Kawashima A, et al. A clinicopathologic study of immunoglobulin G4-related sclerosing disease of the thoracic aorta. J Vasc Surg 2010;52:1587-1595. https://doi.org/10.1016/j.jvs.2010.06.072
  6. Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012;25:1181-1192. https://doi.org/10.1038/modpathol.2012.72
  7. Saeki T, Saito A, Hiura T, et al. Lymphoplasmacytic infiltration of multiple organs with immunoreactivity for IgG4: IgG4-related systemic disease. Intern Med 2006;45:163-167. https://doi.org/10.2169/internalmedicine.45.1431
  8. Bateman AC, Deheragoda MG. IgG4-related systemic sclerosing disease-an emerging and under-diagnosed condition. Histopathology 2009;55:373-383. https://doi.org/10.1111/j.1365-2559.2008.03217.x
  9. Palmisano A, Vaglio A. Chronic periaortitis: a fibro-inflammatory disorder. Best Pract Res Clin Rheumatol 2009;23:339-353. https://doi.org/10.1016/j.berh.2008.12.002
  10. Scheel PJ Jr, Sozio SM, Feeley N. Medical management of retroperitoneal fibrosis. Trans Am Clin Climatol Assoc 2012;123:283-290.

피인용 문헌

  1. A Rare Case of Granulomatosis with Polyangiitis-Related Periaortitis at the Ascending Aorta vol.80, pp.3, 2019, https://doi.org/10.3348/jksr.2019.80.3.537