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Clinical Characteristics and Treatment Outcomes of Cryptogenic Multifocal Ulcerous Stenosing Enteritis in Korea

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Abstract

Background

Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is a rare disease that is characterized by multiple, recurring small intestinal ulcers with stenosis of unknown causes. The aim of this study was to investigate the clinical characteristics and the treatment outcomes of patients with CMUSE in Korea.

Methods

We performed a multicenter study to retrospectively analyze clinical data from 20 patients who suffered from CMUSE between 1984 and 2012. Their clinical characteristics and long-term disease courses were investigated.

Results

The most common initial symptom of CMUSE was abdominal pain (14/20, 70 %). Small bowel series (13/20, 65 %), double-balloon enteroscopy (12/20, 60 %), CT enterography (12/20, 60 %), and capsule endoscopy (10/20, 50 %) were used to diagnose CMUSE. The strictures of the patients were located in the jejunum (5/20, 25 %), ileum (7/20, 35 %), and both jejunum and ileum (6/20, 30 %). The number of patients in a state of remission, persistent disease, and relapse at the end of follow-up were 13/20 (65 %), 2/20 (10 %), and 5/20 (25 %), respectively. The median relapse-free survival was of 67.1 months. Seventy-five percent relapse-free survivals for female and male patients were 93 and 9 months, respectively (P = 0.031).

Conclusion

CMUSE is difficult to diagnose and is an easily relapsing disease. Female patients might have a better prognosis than male patients in terms of the relapse-free time.

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References

  1. Chang DK, Kim JJ, Choi H, et al. Double balloon endoscopy in small intestinal Crohn’s disease and other inflammatory diseases such as cryptogenic multifocal ulcerous stenosing enteritis (CMUSE). Gastrointest Endosc. 2007;66:S96–S98.

    Article  PubMed  Google Scholar 

  2. Freeman HJ. Multifocal stenosing ulceration of the small intestine. World J Gastroenterol. 2009;15:4883–4885.

    Article  PubMed Central  PubMed  Google Scholar 

  3. Choi DH, Jeon SR, Kim JO, et al. Double-balloon enteroscopy in elderly patients: is it safe and useful? Intest Res. 2014;12:313–319.

    Article  PubMed Central  PubMed  Google Scholar 

  4. Perlemuter G, Guillevin L, Legman P, et al. Cryptogenetic multifocal ulcerous stenosing enteritis: an atypical type of vasculitis or a disease mimicking vasculitis. Gut. 2001;48:333–338.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  5. Rocha A, Artigas V. Stenosing ulcerous disease of the jejuno-ileum. Arch Mal Appar Dig Mal Nutr.. 1959;48:1230–1236.

    CAS  PubMed  Google Scholar 

  6. Debray C, Besancon F, Hardouin JP, et al. Cryptogenetic plurifocal ulcerative stenosing enteritis. Arch Mal Appar Dig Mal Nutr. 1964;53:193–206.

    CAS  PubMed  Google Scholar 

  7. Gaucher P, Bigard MA, Champigneulle B, et al. Cryptogenetic multifocal stenosing enteritis: a new case. Gastroenterol Clin Biol. 1985;9:453.

    CAS  PubMed  Google Scholar 

  8. Bokemeyer B, Schmidt FW, Galanski M. Cryptogenic multifocal stenosing enteritis. Z Gastroenterol. 1987;25:745–748.

    CAS  PubMed  Google Scholar 

  9. Perlemuter G, Chaussade S, Soubrane O, et al. Multifocal stenosing ulcerations of the small intestine revealing vasculitis associated with C2 deficiency. Gastroenterology. 1996;110:1628–1632.

    Article  CAS  PubMed  Google Scholar 

  10. Santolaria S, Cabezali R, Ortego J, et al. Diaphragm disease of the small bowel: a case without apparent nonsteroidal antiinflammatory drug use. J Clin Gastroenterol. 2001;32:344–346.

    Article  CAS  PubMed  Google Scholar 

  11. Matsumoto T, Iida M, Matsui T, et al. Non-specific multiple ulcers of the small intestine unrelated to non-steroidal anti-inflammatory drugs. J Clin Pathol. 2004;57:1145–1150.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  12. Spencer H, Kitsanta P, Riley S. Cryptogenic multifocal ulcerous stenosing enteritis. J R Soc Med. 2004;97:538–540.

    Article  PubMed Central  PubMed  Google Scholar 

  13. Chagnon JP, Devars du Mayne JF, Marche C, et al. Multifocal cryptogenetic stenosing enteritis: an autonomous entity? Gastroenterol Clin Biol. 1984;8:808–813.

    CAS  PubMed  Google Scholar 

  14. Doutre LP, Paccalin J, Perissat J, et al. Plurifocal ulcerous stenosing enteritis. A further case. Arch Fr Mal App Dig. 1966;55:537–540.

    CAS  PubMed  Google Scholar 

  15. Faubion WA, Jr., Loftus EV, Jr., Harmsen WS, et al. The natural history of corticosteroid therapy for inflammatory bowel disease: a population-based study. Gastroenterology. 2001;121:255–260.

    Article  CAS  PubMed  Google Scholar 

  16. Lemann M, Mary JY, Colombel JF, et al. A randomized, double-blind, controlled withdrawal trial in Crohn’s disease patients in long-term remission on azathioprine. Gastroenterology. 2005;128:1812–1818.

    Article  CAS  PubMed  Google Scholar 

  17. Fauci AS, Haynes B, Katz P. The spectrum of vasculitis: clinical, pathologic, immunologic and therapeutic considerations. Ann Intern Med. 1978;89:660–676.

    Article  CAS  PubMed  Google Scholar 

  18. Iddan G, Meron G, Glukhovsky A, et al. Wireless capsule endoscopy. Nature. 2000;405:417.

    Article  CAS  PubMed  Google Scholar 

  19. Yamamoto H, Sekine Y, Sato Y, et al. Total enteroscopy with a nonsurgical steerable double-balloon method. Gastrointest Endosc. 2001;53:216–220.

    Article  CAS  PubMed  Google Scholar 

  20. May A, Nachbar L, Wardak A, et al. Double-balloon enteroscopy: preliminary experience in patients with obscure gastrointestinal bleeding or chronic abdominal pain. Endoscopy. 2003;35:985–991.

    Article  CAS  PubMed  Google Scholar 

  21. Baer AN, Bayless TM, Yardley JH. Intestinal ulceration and malabsorption syndromes. Gastroenterology. 1980;79:754–765.

    CAS  PubMed  Google Scholar 

  22. Ruan EA, Komorowski RA, Hogan WJ, et al. Nongranulomatous chronic idiopathic enterocolitis: clinicopathologic profile and response to corticosteroids. Gastroenterology. 1996;111:629–637.

    Article  CAS  PubMed  Google Scholar 

  23. Park JJ, Kim WH, Cheon JH. Outcome predictors for intestinal Behcet’s disease. Yonsei Med J. 2013;54:1084–1090.

    Article  PubMed Central  PubMed  Google Scholar 

  24. Park SJ, Kim WH, Cheon JH. Clinical characteristics and treatment of inflammatory bowel disease: a comparison of Eastern and Western perspectives. World J Gastroenterol. 2014;20:11525–11537.

    Article  PubMed Central  PubMed  Google Scholar 

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Correspondence to Jae Hee Cheon or Dong Kyung Chang.

Additional information

Sook Hee Chung and Sang Un Park have contributed equally to this work.

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Chung, S.H., Park, S.U., Cheon, J.H. et al. Clinical Characteristics and Treatment Outcomes of Cryptogenic Multifocal Ulcerous Stenosing Enteritis in Korea. Dig Dis Sci 60, 2740–2745 (2015). https://doi.org/10.1007/s10620-015-3595-y

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  • DOI: https://doi.org/10.1007/s10620-015-3595-y

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