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Ahmed, Zohair; Rossi, Maria L.; Yong, Sherri; Martin, Daniel K.; Walayat, Saqib; Cashman, Michael; Tsoraides, Steven; Dhillon, Sonu (2016)
Publisher: Co-Action Publishing
Journal: Journal of Community Hospital Internal Medicine Perspectives
Languages: English
Types: Article
Subjects: Behçhet's disease, intestinal, Internal Medicine; Gastroenterology, Beh?het's disease, Internal medicine, Case Report, Behçhet’s disease; Crohn’s disease; inflammatory bowel disease; intestinal; perforation, RC31-1245, perforation, inflammatory bowel disease, Crohn's disease
Behçet’s disease (BD) is a chronic multisystem inflammatory disease most prevalent in Eastern Asia and along the Mediterranean basin, an area referred to as the ‘Silk Road’. The diagnosis of BD is largely based on the International Study Group (ISG) criteria, which are more specific than sensitive. ISG criteria do not include intestinal manifestations, a feature more commonly seen in the West. Intestinal BD is one of several findings that are not typically seen along the ‘Silk Road’. Herein we report a rare case of intestinal BD and compare Western versus traditional BD. A 25-year-old male with a history of painful oral aphthous ulcers, pericarditis, and diffuse papulopustular rash presented to the emergency department with two terminal ileal perforations. Pathology demonstrated mucosal necrosis with active inflammation and no chronic inflammatory changes. Post-surgical laboratory studies showed an elevated c-reactive protein of 35.57 mg/dL, erythrocyte sedimentation rate of 82 mm/h, and a positive anti-Saccharomyces cerevisiae antibody. Rheumatological workup including ANA, RF, PR3 antibody, MPO antibody, ANCA, SSA and SSB, Smith antibody, SCL-70, and anti-Jo-1 antibodies were all negative. His pericarditis symptoms improved with colchicine and prednisone prior to discharge. Our patient did not meet the current ISG criteria for traditional BD; however, he clearly showed findings typically seen in Western patients with BD, which include intestinal manifestations, cardiac involvement, and lack of pathergy reaction and ocular changes. Our investigation demonstrates that the clinical manifestations common to this disorder vary among geographic and ethnic populations. Commonly used criteria for the diagnosis of BD may not be sensitive for some populations, such as Western BD, potentially leading to underdiagnoses and mismanagement. Recognition and select inclusion of these differences may be one way to assist with diagnosing Western BD in the future. As our knowledge of BD continues to evolve, so must the population-specific criteria used to define BD.Keywords: Behçhet’s disease; Crohn’s disease; inflammatory bowel disease; intestinal; perforation(Published: 17 February 2016)Citation: Journal of Community Hospital Internal Medicine Perspectives 2016, 6: 30362 - http://dx.doi.org/10.3402/jchimp.v6.30362
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    • 1. Behc¸et H. U¨ ber rezidivierende, aphtho¨se durch ein Virus verursachte Geschwu¨re am Mund, am Auge und an den Genitalien [Caused about recurrent, aphthous by a virus Ulcers of the mouth, the eyes and genitals]. Dermatol Monatsschr Wochenschr 1937; 105: 1152 7.
    • 2. Azizlerli G, Kose AA, Sarica R, Gul A, Tutkun IT, Kulac M, et al. Prevalence of Behc¸et's disease in Istanbul, Turkey. Int J Dermatol 2003; 42(10): 803 6.
    • 3. Cakir N, Dervis E, Benian O, Pamuk ON, Sonmezates N, Rahimoglu R, et al. Prevalence of Behc¸et's disease in rural western Turkey: a preliminary report. Clin Exp Rheumatol 2004; 22(4 Suppl. 34): 53 5.
    • 4. Yazici H, Fresko I, Yurdakul S. Behc¸et's syndrome: Disease manifestations, management, and advances in treatment. Nat Clin Pract Rheumatol 2007; 3(3): 148 55. doi: http://dx.doi. org/10.1038/ncprheum0436
    • 5. Altenburg A, Mahr A, Maldini C, Kneifel CE, Krause L, Kotter I, et al. Epidemiology and clinical aspects of Adamantiades Behc¸et disease in Gemany. Current data. Ophthalmologe 2012; 109(6): 531 41. doi: http://dx.doi.org/10.1007/s00347-012-2601-4
    • 6. Mumcu G, Inanc N, Aydin SZ, Ergun T, Direskeneli H. Association of salivary S. mutans colonisation and mannosebinding lectin deficiency with gender in Behc¸et's disease. Clin Exp Rheumatol 2009; 27: S32 6.
    • 7. Tojo M, Yanagihori H, Zheng X, Oyama N, Isogai E, Nakamura K, et al. Detection of microbial DNA in skin lesions from patients with Behcet's disease. Adv Exp Med Biol 2003; 528: 185 90.
    • 8. Yokota K, Hayashi S, Araki Y, Isogai E, Kotake S, Yoshikawa K, et al. Characterization of Streptococcus sanguis isolated from patients with Behc¸et's disease. Microbiol Immunol 1995; 39: 729 32.
    • 9. International Study Group for Behc¸et's Disease. Criteria for diagnosis of Behc¸et's disease. Lancet 1990; 335: 1078.
    • 10. Iida M, Kobayashi H, Matsumoto T, Okada M, Guchigami T, Niizeki H, et al. Intestinal Behc¸et disease: Serial changes at radiography. Radiology 1993; 188(1): 65 9.
    • 11. Moon CM, Cheon JH, Shin JK, Jeon SM, Bok HG, Lee JH, et al. Prediction of free bowel perforation in patients with intestinal Behc¸et's disease using clinical and colonoscopic findings. Dig Dis Sci 2010; 55: 2904 11. doi: http://dx.doi.org/ 10.1007/s10620-009-1095-7
    • 12. Davatchi F, Sadeghi AB, Chams DC, Shahram F, Shams H, Nadji A, et al. The saga of diagnostic/classification criteria in Behc¸et's disease. Int J Rheum Dis 2015; 18(6): 594 605. doi: http://dx.doi.org/10.1111/1756-185X.12520
    • 13. Geri G, Wechsler B, Thi Huong du L, Isnard R, Piette JC, Amoura Z, et al. Spectrum of cardiac lesions in Behc¸et disease: A series of 52 patients and review of the literature. Medicine (Baltimore) 2012; 91: 25. doi: http://dx.doi.org/10.1097/MD. 0b013e3182428f49
    • 14. Go¨ ldeli O, Ural D, Komsuog˘lu B, Agacdiken A, Dursun E, Cetinarsian B. Abnormal QT dispersion in Behc¸et's disease. Int J Cardiol 1997; 61: 55.
    • 15. Cheon JH, C¸ elik AF, Kim WH. Behc¸et's disease: Gastrointestinal involvement. In: Yazici Y, Yazici H, eds. Behc¸et's syndrome. 1st ed. New York: Springer; 2010, pp. 165 88.
    • 16. Gu¨ rler A, Boyvat A, Tursen U. Clinical manifestations of Behc¸et's disease. An analysis of 2147 patients. Yonsei Med J 1997; 38: 423 7.
    • 17. Ergun T, Gu¨ rbu¨ z O, Dog˘usoy G, Mat C. Histopathologic features of the spontaneous pustular lesions of Behc¸et's syndrome. Int J Dermatol 1998; 37: 194 6.
    • 4 Citation: Journal of Community Hospital Internal Medicine Perspectives 2016, 6: 30362 - http://dx.doi.org/10.3402/jchimp.v6.30362 (page number not for citation purpose)
    • 18. Dinc A, Karaayvaz M, Caliskaner AZ, Pay S, Erdem H, Turan M. Dermographism and atopy in patients with Behc¸et's disease. J Investig Allergol Clin Immunol 2000; 10: 368.
    • 19. Nussenblatt RB. Uveitis in Behc¸et's disease. Int Rev Immunol 1997; 14: 67.
    • 20. Ideguchi H, Suda A, Takeno M, Ueda A, Ohno S, Ishigatsubo Y. Characteristics of vascular involvement in Behc¸et's disease in Japan: A retrospective cohort study. Clin Exp Rheumatol 2011; 29: 47 53.
    • 21. Yurdakul S, Yazici H, Tu¨ zu¨ n Y, Pazarli H, Yalc¸in B, Altac¸ M, et al. The arthritis of Behc¸et's disease: A prospective study. Ann Rheum Dis 1983; 42: 505 15.
    • 22. Joseph FG, Scolding NJ. Behc¸et's disease in Caucasians: A study of 22 patients. Eur J Neurol 2007; 14: 174 80.
    • 23. Nakae K, Masaki F, Hashimoto T, Inaba G, Mochizuki M, Sakane T. Recent epidemiological features of Behc¸et's disease in Japan. In: Wechsler B, Godeau P, eds. Behc¸et's disease. Amsterdam: Excerpta Medica; 1993, pp. 145 51.
    • 24. Mizuki N, Inoko H, Ohno S. Pathogenic gene responsible for the predisposition in Behc¸et's disease. Int Rev Immunol 1997; 14: 33 48.
    • 25. Ohno S, Ohguchi M, Hirose S, Matsuda H, Wakisaka A, Aizawa M. Close association of HLA-Bw51 with Behc¸et's disease. Arch Ophthalmol 1982; 100: 1455 8.
    • 26. Lehner T. The role of heat shock protein, microbial and autoimmune agents in the aetiology of Behc¸et's disease. Int Rev Immunol 1997; 14: 21 32.
    • 27. Yazici H, Basaran G, Hamuryudan V, Hizli N, Yurdakul S, Mat C, et al. The ten-year mortality in Behc¸et's syndrome. Br J Rheumatol 1996; 35: 139.
    • 28. Hamuryudan V, Melikoglu M. Vascular disease in Behc¸et's syndrome. In: Yazici Y, Yazici H, eds. Behc¸et's syndrome. 1st ed. New York: Springer; 2010, pp. 115 35.
    • 29. Kim ES, Chung WC, Lee KM, Lee BI, Choi H, Han SW, et al. A case of intestinal Behc¸et's disease similar to Crohn's colitis. J Korean Med Sci 2007; 22: 918 22.
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