DOI QR코드

DOI QR Code

A Case of Churg-Strauss Syndrome with Interstinal Perforation

장천공이 동반된 Churg-Strauss 증후군 1예

  • Park, Jung Hoon (Department of Internal Medicine, School of Medicine, Soonchunhyang University) ;
  • Jung, Yun Seok (Department of Internal Medicine, School of Medicine, Soonchunhyang University) ;
  • Kim, Yang Ki (Department of Internal Medicine, School of Medicine, Soonchunhyang University) ;
  • Lee, Young Mok (Department of Internal Medicine, School of Medicine, Soonchunhyang University) ;
  • Hwang, Jung Hwa (Department of Radiology, School of Medicine, Soonchunhyang University) ;
  • Kim, Ki-Up (Department of Internal Medicine, School of Medicine, Soonchunhyang University) ;
  • Kim, Dong Won (Department of Pathology, School of Medicine, Soonchunhyang University) ;
  • Uh, Soo-Taek (Department of Internal Medicine, School of Medicine, Soonchunhyang University) ;
  • Kim, Jae Jun (Department of Surgery, School of Medicine, Soonchunhyang University)
  • 박정훈 (순천향대학교 의과대학 내과학교실) ;
  • 정윤석 (순천향대학교 의과대학 내과학교실) ;
  • 김양기 (순천향대학교 의과대학 내과학교실) ;
  • 이영목 (순천향대학교 의과대학 내과학교실) ;
  • 황정화 (순천향대학교 의과대학 영상의학교실) ;
  • 김기업 (순천향대학교 의과대학 내과학교실) ;
  • 김동원 (순천향대학교 의과대학 병리학교실) ;
  • 어수택 (순천향대학교 의과대학 내과학교실) ;
  • 김재준 (순천향대학교 의과대학 외과학교실)
  • Received : 2009.03.11
  • Accepted : 2009.05.07
  • Published : 2009.05.30

Abstract

Churg-Strauss syndrome (CSS) or allergic granulomatous angiitis is a rare syndrome that is characterized by hypereosinophilic systemic necrotizing vasculitis affecting small- to medium-sized arteries and veins. In general, it occurs in individuals with pre-existing allergic asthma. When CSS appears in patients, it has the following characteristics: eosinophilia of more than 10% in peripheral blood, paranasal sinusitis, pulmonary infiltrates, histological proof of vasculitis with extravascular eosinophils, and mononeuritis multiplex or polyneuropathy. Therapeutic trials dedicated to Churg-Strauss syndrome have been limited due to the rarity of this disorder and the difficulty in making a histological diagnosis. Proper treatment of patients with CSS is not widely known. In this case study, we report on our experience with an unusual patient case, characterized by purpura and a perforation of the small intestine after inadequate steroid therapy.

천식조절 중이던 환자에서 임상증상과 조직검사를 바탕으로 진단된 Churg-Strauss 증후군을 진단하고 치료 중 발생한 장천공을 경험하였기에 문헌고찰과 함께 보고하는 바이다.

Keywords

References

  1. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27:277-301
  2. Keogh KA, Specks U. Churg-Strauss syndrome: update on clinical, laboratory and therapeutic aspects. Sarcoidosis Vasc Diffuse Lung Dis 2006;23:3-12
  3. Kurita M, Niwa Y, Hamada E, Hata Y, Oshima M, Mutoh H, et al. Churg-Strauss syndrome (allergic granulomatous angiitis) with multiple perforating ulcers of the small intestine, multiple ulcers of the colon, and mononeuritis multiplex. J Gastroenterol 1994;29:208-13 https://doi.org/10.1007/BF02358685
  4. Jung SH, Kim KH, Nam SM, Park HC, Chu HK, Whang IS, et al. A case of Churg-Strauss syndrome with manifestations of esophageal ulcer, acute acalculous cholecystitis and ischemic colitis. Korean J Med 1993;45:369-75
  5. Kim JH, Yoon KY, Shin YM. Churg-Strauss syndrome with multiple small bowel perforation. J Korean Surg Soc 1998;54:148-52
  6. Kim YB, Choi SW, Park IS, Han JY, Hur YS, Chu YC. Churg-Strauss syndrome with perforating ulcers of the colon. J Korean Med Sci 2000;15:585-8 https://doi.org/10.3346/jkms.2000.15.5.585
  7. Oh MJ, Ji SH, Lim DH, Min TH, Lee BJ, Choi DC, et al. A case of intestinal perforation in Churg-Strauss syndrome patient. Korean J Med 2004;67:208-12
  8. Lee YK, Choi KW, Lee CY, Kim Hs, Kim KH, Kim YH. A case of Churg-Strauss syndrome associated with small bowel perforation following high dose systemic steroid intravenous injection. Korean J Dermatol 2008;46:950-4
  9. Vaglio A, Martorana D, Maggiore U, Grasselli C, Zanetti A, Pesci A, et al. HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome. Arthritis Rheum 2007;56:3159-66 https://doi.org/10.1002/art.22834
  10. Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore) 1984;63:65-81
  11. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American college of rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990;33:1094-100 https://doi.org/10.1002/art.1780330806
  12. Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome: clinical study and long-term follow-up of 96 patients. Medicine (Baltimore) 1999;78:26-37 https://doi.org/10.1097/00005792-199901000-00003
  13. Chumbley LC, Harrison EG Jr, DeRemee RA. Allergic granulomatosis and angiitis (Churg-Strauss syndrome): report and analysis of 30 cases. Mayo Clin Proc 1977;52:477-84
  14. Kaneki T, Kawashima A, Hayano T, Honda T, Kubo K, Koizumi T, et al. Churg-Strauss syndrome (allergic granulomatous angitis) presenting with ileus caused by ischemic ileal ulcer. J Gastroenterol 1998;33:112-6 https://doi.org/10.1007/s005350050054
  15. Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome: a prospective study in 342 patients. Medicine (Baltimore) 1996;75:17-28 https://doi.org/10.1097/00005792-199601000-00003

Cited by

  1. A Case of Recurrent Bowel Perforation in a Patient with Churg-Strauss Syndrome vol.17, pp.3, 2010, https://doi.org/10.4078/jkra.2010.17.3.326
  2. Acute Extensive Ischemic Enteritis in a Young Man Diagnosed with Wireless Capsule Endoscopy: A Case Report vol.61, pp.3, 2009, https://doi.org/10.4166/kjg.2013.61.3.160