A Case of Churg-Strauss Syndrome Manifesting as Colonic Aphthous Ulcers with Granuloma

대장의 아프타성 궤양 및 점막 육아종으로 발현된 Churg-Strauss 증후군 1예

  • Park, Jun-Sup (Department of Internal Medicine, Ilsan Paik Hospital, Inje University College of Medicine) ;
  • Kim, Nam-Hoon (Department of Internal Medicine, Ilsan Paik Hospital, Inje University College of Medicine) ;
  • Park, Joo-Hyun (Department of Internal Medicine, Ilsan Paik Hospital, Inje University College of Medicine) ;
  • Bae, Won-Ki (Department of Internal Medicine, Ilsan Paik Hospital, Inje University College of Medicine) ;
  • Jeong, Jae-Won (Department of Internal Medicine, Ilsan Paik Hospital, Inje University College of Medicine) ;
  • Lee, June-Sung (Department of Internal Medicine, Ilsan Paik Hospital, Inje University College of Medicine) ;
  • Moon, Young-Soo (Department of Internal Medicine, Ilsan Paik Hospital, Inje University College of Medicine) ;
  • Joo, Mee (Department of Pathology, Ilsan Paik Hospital, Inje University College of Medicine)
  • 박준섭 (인제대학교 의과대학 일산백병원 내과학교실) ;
  • 김남훈 (인제대학교 의과대학 일산백병원 내과학교실) ;
  • 박주현 (인제대학교 의과대학 일산백병원 내과학교실) ;
  • 배원기 (인제대학교 의과대학 일산백병원 내과학교실) ;
  • 정재원 (인제대학교 의과대학 일산백병원 내과학교실) ;
  • 이준성 (인제대학교 의과대학 일산백병원 내과학교실) ;
  • 문영수 (인제대학교 의과대학 일산백병원 내과학교실) ;
  • 주미 (인제대학교 의과대학 일산백병원 병리학교실)
  • Published : 2011.07.30

Abstract

Churg-Strauss syndrome is characterized by asthma, peripheral and tissue eosinophilia, extravascular granuloma formation, and vasculitis of multiple organ systems. The gastrointestinal tract may be involved in 20% to 50% of patients, mainly with abdominal pain, diarrhea and GI bleeding. Ulcers in the small and large bowel are an uncommon manifestation, usually detected during laparotomy performed in the setting of bowel perforation. Endoscopically proven segmental colonic aphthous ulcers with typical pathologic findings such as extravascular granuloma formation, eosinophilic infiltration, or vasculitis are a rarer manifestation. We present a patient with Churg-Strauss syndrome and two aphthous ulcers in the sigmoid colon that healed after treatment with high dose steroids and cyclophosphamide.

알레르기성 육아종증은 기관지 천식, 부비동염, 말초혈액 및 조직 내 호산구 증가, 말초신경병증 등을 특징으로 하는 전신성 혈관염으로 비교적 드문 질환이다. 이 질환은 폐, 심장, 소화기, 피부, 근육 신경계, 신장 등 다양한 장기를 침범한다. 복통과 설사의 증상이 심하여 원인을 찾기위해 시행한 대장내시경검사에서 구불결장에서 국소적으로 2개의 아프타성 궤양이 관찰되었다. 증상에 비해 미미한 내시경 소견이었으나 병리조직검사에서 혈관 외부의 육아종 형성 및 혈관염 소견을 확인하였고 이로 인해 알레르기성 육아종증을 진단하는 계기가 되었다. 동반된 기관치 천식, 말초신경병증, 부비동염 및 말초 호산구 증가증을 통해 알레르기성 육아종증을 확진하였고 스테로이드 사용을 통하여 복통 및 설사의 증상의 개선을 보여 문헌고찰과 함께 보고한다.

Keywords

References

  1. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27:277-301.
  2. Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome. Clinical study and longterm follow-up of 96 patients. Medicine (Baltimore) 1999;78:26-37. https://doi.org/10.1097/00005792-199901000-00003
  3. Oh MJ, Ji SH, Lim DH, et al. A case of intestinal perforation in Churg-Strauss syndrome patient. Korean J Med 2004;67:208-212.
  4. Kim JH, Yoon KY, Shin YM. Churg-Strauss syndrome with multiple small bowel perforation: a case report. J Korean Surg Soc 1998;54:148-152.
  5. 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;15:585-588. https://doi.org/10.3346/jkms.2000.15.5.585
  6. Kim TG, Noh SY, Lee YJ, et al. A case of Churg-Strauss syndrome with appendicitis and lower gastrointestinal bleeding. Korean J Gastrointest Endosc 1999;19:977-982.
  7. Paik CH, Chung YW, Chun SJ, et al. A case of Churg-Strauss syndrome with multiple colonic ulcers. Korean J Gastrointest Endosc 2002;24:171-175.
  8. Shimamoto C, Hirata I, Ohshiba S, Fujiwara S, Nishio M. Churg-Strauss syndrome (allergic granulomatous angiitis) with peculiar multiple colonic ulcers. Am J Gastroenterol 1990;85:316-319.
  9. Kang YK, Song SY, Kim WH, Kim YI. Gastrointestinal cytomegalovirus infection: a clinicopathologic analysis of 8 cases. Korean J Pathol 1994;28:22-29.
  10. Lee SY. Cytomegalovirus colitis in an immunocompetent adult. Korean J Gastroenterol 2007;50:75-77.
  11. Nagar AB. Isolated colonic ulcers: diagnosis and management. Curr Gastroenterol Rep 2007;9:422-428. https://doi.org/10.1007/s11894-007-0053-9
  12. Ona FV, Allende HD, Vivenzio R, Zaky DA, Nadaraja N. Diagnosis and management of nonspecific colon ulcer. Arch Surg 1982;117:888-894. https://doi.org/10.1001/archsurg.1982.01380310014004
  13. Camilleri M, Pusey CD, Chadwick VS, Rees AJ. Gastrointestinal manifestations of systemic vasculitis. Q J Med 1983;52:141-149.
  14. Chumbley LC, Harrison EG Jr, DeRemee RA. Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases. Mayo Clin Proc 1977;52:477-484.
  15. 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. https://doi.org/10.1097/00005792-198403000-00001
  16. Leen EJ, Rees PJ, Sanderson JD, Wilkinson ML, Filipe MI. A case of Churg-Strauss syndrome: tissue diagnosis established by sigmoidoscopic rectal biopsy. Gut 1996;38:299-301. https://doi.org/10.1136/gut.38.2.299
  17. Memain N, De BM, Guillevin L, Wechsler B, Meyer O. Delayed relapse of Churg-Strauss syndrome manifesting as colon ulcers with mucosal granulomas: 3 cases. J Rheumatol 2002;29:388-391.