Поражение кишечника при болезни Бехчета

Голоева Р.Г., Алекберова З.С., Лисицына Т.А. и др. Поражение кишечника при болезни Бехчета. Терапевтический архив. 2019; 91 (5): 111–119. DOI: 10.26442/00403660.2019.05.000247

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Goloeva R.G., Alekberova Z.S., Lisitsyina T.A., et al. Therapeutic Archive. 2019; 91 (5): 111–119. DOI: 10.26442/00403660.2019.05.000247

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Аннотация

Болезнь Бехчета (ББ) – системный васкулит неизвестной этиологии, характеризуется рецидивами язвенного процесса в ротовой полости и гениталиях, поражением глаз, суставов, сосудов и других органов. Тяжесть и прогноз ББ определяет органная патология. Среди последней поражение кишечника наименее изучено. Его верификация при ББ затруднена многообразием клинических проявлений, их схожестью с воспалительными заболеваниями кишечника, отсутствием информативных лабораторных тестов, патогномоничных эндоскопических и гистологических признаков. Поражение кишечника при ББ (ПКББ) может привести к серьезным осложнениям (массивное кровотечение, перфорация кишки и образование свища), что может не только существенно снизить качество жизни пациента, но и стать причиной летального исхода. Лечение ПКББ не стандартизовано, в основном носит эмпирический характер и проводится курсами. Цель терапии – достижение клинической ремиссии, заживление язв кишечника, предотвращения хирургического вмешательства. В статье представлен случай тяжелого рефрактерного поражения кишечника у пациента с ББ, потребовавший дважды неотложной хирургической помощи – удаление половины, а затем и всей толстой кишки из-за множественных перфораций. Приведен кратко обзор литературы и обсуждены диагностические сложности ПКББ.

Ключевые слова: болезнь Бехчета, язвы кишечника, перфорация кишки, колоноскопия, ультразвуковое исследование кишечника, гемиколэктомия.

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Behçet's disease (BD) is a systemic vasculitis of unknown origin, characterized by recurrences of the ulcerative process in the oral cavity and on the genitals, inflammatory damage of the eyes, joints, vessels and other organs. The severity and prognosis of BD determines organ pathology. Intestinal manifestations of BD (intestinal BD) are the least studied. Its verification in BD is complicated by the variety of clinical manifestations, their similarity with inflammatory bowel diseases, the lack of informative laboratory tests, pathognomonic endoscopic and histological signs. Intestinal BD can lead to serious complications (massive bleeding, intestinal perforation and fistula formation), which can not only significantly reduce the quality of the patient’s life, but also cause death. Treatment of intestinal BD is not standardized; it is mainly empirical and conducted courses. The purpose of therapy is to achieve clinical remission, healing of intestinal ulcers and prevention of surgery. The article presents a case of severe refractory intestinal BD, requiring twice emergency surgical care – removal of half and then the whole of the colon because of multiple perforations. A brief review of the literature is given and diagnostic difficulties of intestinal BD are discussed.

Keywords: Behçet's disease, intestinal ulcers, intestinal perforation, colon-oscopy, intestinal ultrasound, hemicolectomy.

Список литературы

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9. Greco A, De Virgilio A, Ralli M, et al. Behçet's disease: New insights into pathophysiology, clinical features and treatment options. Autoimmun Rev. 2018;17(6):567-75.
10. Yazici Y, Yurdakul S, Yazici H. Behcet’s syndrome. Curr Rheumatol Rep. 2010;12:429-35.
11. Choi IJ, Kim JS, Cha SD, et al. Long-term clinical course and prognostic factors in intestinal Behçet's disease. Dis Colon Rectum. 2000;43:692-700.
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21. Marin A, Tribus L, Fierbinteanu-Braticevici C. The importance of intestinal ultrasound and elastographic techniques in inflammatory bowel diseases. Med Ultrason. 2018;20(2):228-36.
22. Cheon JH, Han DS, Park JY, et al. Development, validation, and responsive-ness of a novel disease activity index for intestinal Behcet’s disease. Inﬂamm Bowel Dis. 2011;17:605-13.
23. Kobayashi K, Ueno F, Bito S. Development of consensus statements for the diagnosis and management of intestinal Behçet’s disease using a modified Delphi approach. J Gastroenterol. 2007;42:737-45.
24. Houman H, Ben Dahmen F, Ben Ghorbel I, et al. Behçet's disease associated with Crohn's disease. Ann Med Interne (Paris). 2001;152(7):480-2.
25. Kallinowski B, Nöldge G, Stiehl A. Crohn's disease with Behçet's syndrome like appearance: a case report. Z Gastroenterol. 1994;32(11):642-4.
26. Lee SK, Kim BK, Kim TI, et al. Differential diagnosis of intestinal Behçet's disease and Crohn's disease by colonoscopic findings. Endoscopy. 2009;41(1):9-16.
27. Zhu Z, Shu X, Long S, et al. Ulcerative colitis followed by the development of typical intestinal Behçet disease: A case report. Medicine (Baltimore). 2018;97(7):9882.
28. Hisamatsu T, Ueno F, Matsumoto T, et al. The 2nd edition of consensus statements for the diagnosis and management of intestinal Behçet’s disease: indication of anti-TNFα monoclonal antibodies. J Gastroenterol. 2014;49:156-62.
29. Hatemi G, Christensen R, Bang D, et al. Ann Rheum Dis. 2018;77:808-18.
30. Yazici H, Esen F. Mortality in Behçet's syndrome. Clin Exp Rheumatol. 2008;26(5 Suppl 51):S138-40. Review.
31. Park YE, Cheon JH, Park J. The outcomes and risk factors of early reoperation after initial intestinal resective surgery in patients with intestinal Behçet's disease. Int J Colorectal Dis. 2017;32(4):591-4.
32. Hisamatsu T, Hayashida M. Treatment and outcomes: medical and surgical treatment for intestinal Behçet's disease. Intest Res. 2017;15(3):318-27.

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1. Alekberova ZS. Bolezn' Bekhcheta (Behcet’s disease)]. Moscow, 2007:90 p. (In Russ.).
2. Jennette J, Falk R, Bacon P, et al. 2012 revised international Chapell Hill Consensus conference nomenclature of vasculitides. Arthritis Rheumatism. 2013;65(1):1-11.
3. Criteria for diagnosis of Behcet’s, disease. International Study Group for Behcet’s Disease. Lancet. 1990;335:1078-80.
4. International Team for the Revision of the international criteria for Behcet`s disease (ICBD) collaborative study 27 countries on the new criteria. J Eur Acad Dermatol Venerol. 2014;28(3):338-47.
5. Rheumatology. Russian clinical guidelines]. Moscow: GEOTAR-Media, 2017:464 p. (In Russ.).
6. Hatemi I, Esatoglu S, Hatemi G, et al. Characteristics, Treatment, and Long-Term Outcome of Gastrointestinal Involvement in Behcet’s Syndrome A Strobe-Compliant Observational Study From a Dedicated Multidisciplinary Center. 2016;95(16):33-48.
7. Lennikov A, Alekberova Z, Goloeva R, et al. Single center study on ethnic and clinical features of Behcet’s disease in Moscow, Russia. Clin Rheumatol. 2015;34:321-7.
8. O'Duffy JD. Suggested criteria for diagnosis of Behcet's disease. J Rheumatol. 1974:1(l):18(abstr).
9. Greco A, De Virgilio A, Ralli M, et al. Behçet's disease: New insights into pathophysiology, clinical features and treatment options. Autoimmun Rev. 2018;17(6):567-75.
10. Yazici Y, Yurdakul S, Yazici H. Behcet’s syndrome. Curr Rheumatol Rep. 2010;12:429-35.
11. Choi IJ, Kim JS, Cha SD, et al. Long-term clinical course and prognostic factors in intestinal Behçet's disease. Dis Colon Rectum. 2000;43:692-700.
12. Jung YS, Yoon JY, Hong SP, et al. Influence of age at diagnosis and sex on clinical course and long-term prognosis of intestinal Behçet's disease. Inflamm Bowel Dis. 2012;18:1064-71.
13. Glosemeyer R, Deppe H, Dormann A, et al. Ulcerative esophagitis and colitis as rare manifestations of Adamantiades-Behcet disease. Z Gastroenterol. 2001;39(2):167-71.
14. Chong S, Wright V, Nishigame T, et all. Infantile colitis: manifestation of intestinal Behcet's syndrome. J Pediatr Gastroenterol Nutr. 1988;7(4):622-7.
15. Jin A. The intestinal manifestation of Behçet's disease: report of 16 cases. Zhonghua Nei Ke Za Zhi. 1982;21(12):725-7, 776.
16. Lee HJ, Cheon JH. Optimal diagnosis and disease activity monitoring of intestinal Behçet's disease. Intest Res. 2017;15(3):311-17.
17. Yim SM, Kim DH, Lee HJ, et al. Mucosal healing predicts the long-term prognosis of intestinal Behçet'sdisease. Dig Dis Sci. 2014;59 (10):2529-35.
18. Lee CR, Kim WH, Cho YS, et al. Colonoscopic findings in intestinal Behçet's disease. Inflamm Bowel Dis. 2001;7(3):243-9.
19. Samsonova TV, Orlova LP. Ultrazvukovaya semiotikabolezni Krona tonkoy kishki. Koloproktologia. 2014;1:60-8 (In Russ.).
20. Ivashkin VT, Sheligin UA, Halif BL. i dr. Klinicheskie rekomendatsii Rossiyskoi gastroenterologicheskoi associacii I associacii koloproktologov Rossii po diagnostike b lecheniu bolesni Krona. Koloproktologia. 2017;2(60):7-30 (In Russ.).
21. Marin A, Tribus L, Fierbinteanu-Braticevici C. The importance of intestinal ultrasound and elastographic techniques in inflammatory bowel diseases. Med Ultrason. 2018;20(2):228-36.
22. Cheon JH, Han DS, Park JY, et al. Development, validation, and responsive-ness of a novel disease activity index for intestinal Behcet’s disease. Inﬂamm Bowel Dis. 2011;17:605-13.
23. Kobayashi K, Ueno F, Bito S. Development of consensus statements for the diagnosis and management of intestinal Behçet’s disease using a modified Delphi approach. J Gastroenterol. 2007;42:737-45.
24. Houman H, Ben Dahmen F, Ben Ghorbel I, et al. Behçet's disease associated with Crohn's disease. Ann Med Interne (Paris). 2001;152(7):480-2.
25. Kallinowski B, Nöldge G, Stiehl A. Crohn's disease with Behçet's syndrome like appearance: a case report. Z Gastroenterol. 1994;32(11):642-4.
26. Lee SK, Kim BK, Kim TI, et al. Differential diagnosis of intestinal Behçet's disease and Crohn's disease by colonoscopic findings. Endoscopy. 2009;41(1):9-16.
27. Zhu Z, Shu X, Long S, et al. Ulcerative colitis followed by the development of typical intestinal Behçet disease: A case report. Medicine (Baltimore). 2018;97(7):9882.
28. Hisamatsu T, Ueno F, Matsumoto T, et al. The 2nd edition of consensus statements for the diagnosis and management of intestinal Behçet’s disease: indication of anti-TNFα monoclonal antibodies. J Gastroenterol. 2014;49:156-62.
29. Hatemi G, Christensen R, Bang D, et al. Ann Rheum Dis. 2018;77:808-18.
30. Yazici H, Esen F. Mortality in Behçet's syndrome. Clin Exp Rheumatol. 2008;26(5 Suppl 51):S138-40. Review.
31. Park YE, Cheon JH, Park J. The outcomes and risk factors of early reoperation after initial intestinal resective surgery in patients with intestinal Behçet's disease. Int J Colorectal Dis. 2017;32(4):591-4.
32. Hisamatsu T, Hayashida M. Treatment and outcomes: medical and surgical treatment for intestinal Behçet's disease. Intest Res. 2017;15(3):318-27.

Авторы

Р.Г. Голоева 1, З.С. Алекберова 1, Т.А. Лисицына 1, Е.А. Степанова 2, Л.П. Орлова 3

1 ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой», Москва, Россия;
2 ГБУЗ «Городская клиническая больница им. В.М. Буянова» Департамента здравоохранения города Москвы, Москва, Россия;
3 ФГБУ «Государственный научный центр колопроктологии им. А.Н. Рыжих» Минздрава России, Москва, Россия

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