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Behçet’s disease

Year 2011, Volume: 1 Issue: 1, 65 - 73, 31.01.2014

Abstract

Behcet’s Disease (BD) is a chronic multisystem vascularinflammatory disorder, firstly described by the Turkish dermatologist Hulusi Behçet (1889-1948) in 1937. It is thought to be auto-immune and characterized by attacks of oral and genital ulcers, arthritis, cutaneous lesions, ocular lesions, involvement of the gastrointestinal tract and central nervous system. However the etiology of BD is still unknown, infectious, psychological, genetic and immunological factors can be counted as the possible etiologic factors. This review updates the BD in point of epidemiology, clinical symptoms, diagnosis, prognosis and etiopathology.

Key words: Behçet’s disease, epidemiology, etiopathology

References

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  • Evereklioglu C: Current concepts in the etiology and treatment of Behcet disease. Surv Ophthalmol 2005;50:297-350.
  • Karlidag R, Unal S, Evereklioglu C, Sipahi B, Er H, Yologlu S. Stressful life events, anxiety, depression and coping mechanisms in patients with Behcet’s disease. J Eur Acad Dermatol Venereol 2003;17:670-675.
  • Al-Otaibi LM, Porte SR, Poite TWJ: Behçet’s Disease: A Review. J Dent Res, 2005; 84: 209-222.
  • Pamuk ÖN, Çakır N. Behçet hastalığı epidemiyolojisi. T Klin J Int Med Sci 2005;1:3-9.
  • Rhee SH, Kim YB, Lee ES: Comparison of Behçet’s Disease and Recurrent Aphthous Ulcer According to Characteristics of Gastrointestinal Symptoms. J Korean Med Sci 2005; 20; 971-976.
  • Davatchi F, Shahram F, Akbarian M, Gharibdoost F, Chams C, Chams H, Mansoori P, Nadji A: Classification tree for the diagnosis of Behcet’s disease. In: Wechsler B, Godeau P (eds): Behcet’s disease. Excerpta Medica. Amsterdam, 1993; 245-248.
  • Cakir N, Dervis E, Benian O: Prevalence of Behcet’s disease in rural western Turkey: a preliminary report. Clin Exp Rheumatol 2004; 22: 53-5.
  • Kotake S, Namba K, Higashi K: The change of clinical manifestations of patients with Behcet’s disease in Japan. Adv Exp Med Biol 2003; 528:83-84.
  • Saylan T, Mat C, Fresko I, Melikoğlu M: Behçet’s disease in the Middle East. Clinics in Dermatology 1999; 17: 209-223.
  • Al-Dalaan AN, al Balaa SR, el Ramahi K, al-Kawi Z, Bohlega S, Bahabri S, al Janadi MA. Behçet’s disease in Saudi Arabia. J Rheumatol. 1994;21:658-61.
  • Al-Fahad SA, Al-Araji AH: Neuro-Behcet’s disease in Iraq: a study of 40 patients. Journal of the Neurological Sciences, 1999; 170: 105-111.
  • Marshall SE: Behçet’s disease. Best Practice & Research Clinical Rheumatology 2004;18: 291-311.
  • Demirhindi O, Yazıcı H, Binyıldız P, ve ark. Silivri fener köyü ve yöresinde Behçet hastalığı sıklığı ve bu hastalığın toplum içinde taranmasında kullanılabilecek bir yöntem. Cerrahp Tıp Fak Derg. 1981;12:509-514.
  • Yurdakul S, Yazici H, Tuzun Y, ve ark. THA arthritis of Behcet’s disease: a prospective study. Ann Rheum Dis 1983; 42: 505-515.
  • Gul A, Inanc M, Ocal L: Familial aggregation of Behcet’s disease in Turkey. Ann Rheum Dis 2000; 59:622-625.
  • Yazici H, Fresko I, Tunc R: Behcet’s syndrome: pathogenesis, clinical manifestations and treatment in Vasculitis by Gene V. Ball, S. Louis Bridges ed. Oxford University Press, USA; 2002;1:406-32.
  • Tüzün Y, Yurdakul S, Mat C, Özyazgan Y, Hamuryudan V, Tüzün B, Yazıcı H: Epidemiology of Behçet’s Syndrome in Turkey. International Journal of Dermatology 1996; 35: 618-620.
  • Gul A, Esin S, Dilsen N: Immunohistology of skin pathergy reaction in Behçet’s disease. British Journal of Dermatology 1995; 132: 901-907.
  • Demirkesen C, Tuzuner N, Mat C: Clinicopathologic evaluation of nodular cutaneous lesions of Behcet syndrome. Am J Clin Pathol 2001; 116: 341-346.
  • Saricaoglu H, Karadogan SK, Bayazit N, Yucel A, Dilek K, Tunali S. Clinical features of late onset Behcet’s disease: report of nine cases. Int J Dermatol 2006;45:1284-1287.
  • Calamia KT, Wilson FC, Icen M, Crowson CS, Gabriel SE, Kremers HM: Epidemiology and clinical characteristics of Behçet’s disease in the US: a population based study.Arthritis Rheum. 2009;61:600-604.
  • Yaylalı GF: Denizli ve Çevresinde Ailesel Akdeniz Ateşi ve Behçet Hastalığı Prevalansı: “Sıfır Hasta” Modelinin uygulanması. Uzmanlık tezi. Denizli, 2003.
  • Ando K, Fujino Y, Hijikata K: Epidemiological features and visual prognosis of Behçet’s disease. Jpn J Ophthalmol 1999;43: 312-317.
  • Bayraktar Y, Balkanci F, Bayraktar M: Budd-Chiari syndrome: a common complication of Behcet’s disease. Am J Gastroenterol 1997; 92: 858-86.
  • Benezra D, Cohen E: Treatment and visual prognosis in Behçet’s disease. Br J Ophthalmol 1986;70: 589-592.
  • Sakamoto M, Akazawa K, Nishioka Y: Prognostic factors of vision in patients with Behcet disease. Ophthalmology 1995;102:317-21.
  • Kural-Seyahi E, Fresko I, Seyahi N: The long-term mortality and morbidity of Behcet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore). 2003; 82:60-76.
  • Karaca M :Ailevi Behçet Hastalığı Olgularında Hedef Organ İlişkilerinin Faktör Analizi İle İncelenmesi. Uzmanlık Tezi, İstanbul, 2008.
  • Zouboulis C.C: Epidemiology of Adamantiades-Behcet’s disease. Ann Med Interne 1999; 150: 488-498.
  • Gurler A, Boyvat A, Tursen U: Clinical manifestations of Behcet’s disease: an analysis of 2147 patients. Yonsei Med J 1997; 38: 423-427.
  • Yazici H, Fresko I: Behcet’s disease and other autoinflammatory conditions: what’s in a name? Clin Exp Rheumatol 2005; 23 : 1-2.
  • Ilknur T, Pabuccuoglu U, Akin C, Lebe B, Gunes AT. Histopathologic and direct immunofluorescence findings of the papulopustular lesions in Behcet’s disease. Eur J Dermatol 2006;16:146-150.
  • Chang HK, Kim JU, Cheon KS, Chung HR , Lee KW, Lee IH: HLA-B51 and its allelic types in association with Behçet’s disease and recurrent aphthous stomatitis in Korea. Clin Exp Rheumatol. 2001 ; 19: 31-35.
  • Yıldırım M, Kılınç Y, Ceyhan MA. Behçet hastalığı patogenezindeki yenilikler S.D.Ü.Tıp Fak.Derg. 2009;16:29-34.
  • Kılınç Y: Behçet Hastalarında Yaşam Kalitesi Anksiyete ve Depresyon, Uzmanlık Tezi, Isparta, 2007.
  • Aslan SH, Soylu MB, Alparslan ZN, Ünal M: Behçet Hastalığında psikososyal etkenler ve ruhsal bulgular. Türk Psikiyatri Dergisi 1996;1:215-221.
  • Yazici H, Chamberlain MA, Schreuder I: HLA antigens in Behcet’s disease: a reappraisal by a comparative study of Turkish and British patients. Ann Rheum Dis. 1980 ;39:344-348.
  • Ohno S, Asanuma T, Sugiura S: HLA-Bw51 and Behcet’s disease. JAMA 1978; 240: 529.
  • Celenk C, Aydin F, Unsal M: Pulmonary alterations in Behcet’s disease. Europan Journal of Radiology, 2009;70:317-319.
  • Yazici H, Chamberlain MA, Schreuder GM: HLA B5 and Behcet’s disease. Ann Rheum Dis. 1983;42:602-603.
  • Verity DH, Marr JE, Ohno S: Behcet’s disease, the Silk Road and HLA- B51: historical and geographical perspectives. Tissue Antigens. 1999; 54: 213-220.
  • Gul A, Uyar FA, Inanc M: Lack of association of HLA-B*51 with a severe disease course in Behcet’s disease. Rheumatology 2001; 40: 668-672.
  • Muftuoglu AU, Yazici H, Yurdakul S: Behcet’s disease: lack of correlation of clinical manifestations with HLA antigens. Tissue Antigens. 1981; 17: 226-230.
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  • Turan B, Gallati H, Erdi H, Gürler A, Michel BA, Villiger PM: Systemic levels of the T cell regulatory cytokines IL-10 and IL-12 in Behçet’s disease; soluble TNFR-75 as a biological marker of disease activity. J Rheumatol. 1997 ; 24 : 128-132.
  • Akman-Demir G, Tüzün E, Içöz S, Yeşilot N, Yentür SP, Kürtüncü M, Mutlu M, Saruhan-Direskeneli G: Interleukin-6 in neuro-Behçet’s disease: association with disease subsets and long-term outcome. Cytokine, 2008; 44: 373-376.
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  • Mochizuki M, Morita E, Yamamoto S, Yamana S: Characteristics of T cell lines established from skin lesions of Behcet’s disease. Journal of Dermatological Science 1997; 15: 9-13.
  • Hatemi G, Bahar H, Uysal S: The pustular skin lesions in Behcet’s syndrome are not sterile. Ann Rheum Dis 2004; 63: 1450-1452.
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  • Alpsoy E, Durusoy C, Yilmaz E: Interferon alfa-2a in the treatment of Behcet disease: a randomized placebo-controlled and double-blind study. Arch Dermatol. 2002 ;138:467-471.
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Behçet hastalığı

Year 2011, Volume: 1 Issue: 1, 65 - 73, 31.01.2014

Abstract

Behçet Hastalığı (BH) ilk olarak 1937 yılında Türk Dermatolog Hulusi Behçet (1889-1948) tarafından tanımlanan kronik vasküler- enflamatuar multisistemik bir hastalıktır. Oto-immün olduğu düşünülen hastalık, ataklarla seyreden oral ve genital aftlar, artrit, kutanöz lezyonlar, göz lezyonları, gastrointestinal sistem ve merkezi sinir sistem tutulumuyla karakterizedir. BH’nin etyolojisi günümüzde hala bilinmemekle birlikte olası etyolojik faktörler arasında enfeksiyöz, psikolojik, genetik ve immünolojik faktörler sayılabilmektedir. Bu derleme BH’yi epidemiyolojik, klinik belirtiler, diagnoz, prognoz ve etyopatogenetik bakımdan güncellemektedir.

Anahtar Kelimeler: Behçet hastalığı, epidemiyoloji, etyopatogenez

References

  • Scully C, Gorsky M, Lozada-Nur F: The Diagnosis and management of recurrent aphthous stomatitis: a consensus approach. J Am Dent Assoc. 2003; 134: 200-207.
  • Evereklioglu C: Current concepts in the etiology and treatment of Behcet disease. Surv Ophthalmol 2005;50:297-350.
  • Karlidag R, Unal S, Evereklioglu C, Sipahi B, Er H, Yologlu S. Stressful life events, anxiety, depression and coping mechanisms in patients with Behcet’s disease. J Eur Acad Dermatol Venereol 2003;17:670-675.
  • Al-Otaibi LM, Porte SR, Poite TWJ: Behçet’s Disease: A Review. J Dent Res, 2005; 84: 209-222.
  • Pamuk ÖN, Çakır N. Behçet hastalığı epidemiyolojisi. T Klin J Int Med Sci 2005;1:3-9.
  • Rhee SH, Kim YB, Lee ES: Comparison of Behçet’s Disease and Recurrent Aphthous Ulcer According to Characteristics of Gastrointestinal Symptoms. J Korean Med Sci 2005; 20; 971-976.
  • Davatchi F, Shahram F, Akbarian M, Gharibdoost F, Chams C, Chams H, Mansoori P, Nadji A: Classification tree for the diagnosis of Behcet’s disease. In: Wechsler B, Godeau P (eds): Behcet’s disease. Excerpta Medica. Amsterdam, 1993; 245-248.
  • Cakir N, Dervis E, Benian O: Prevalence of Behcet’s disease in rural western Turkey: a preliminary report. Clin Exp Rheumatol 2004; 22: 53-5.
  • Kotake S, Namba K, Higashi K: The change of clinical manifestations of patients with Behcet’s disease in Japan. Adv Exp Med Biol 2003; 528:83-84.
  • Saylan T, Mat C, Fresko I, Melikoğlu M: Behçet’s disease in the Middle East. Clinics in Dermatology 1999; 17: 209-223.
  • Al-Dalaan AN, al Balaa SR, el Ramahi K, al-Kawi Z, Bohlega S, Bahabri S, al Janadi MA. Behçet’s disease in Saudi Arabia. J Rheumatol. 1994;21:658-61.
  • Al-Fahad SA, Al-Araji AH: Neuro-Behcet’s disease in Iraq: a study of 40 patients. Journal of the Neurological Sciences, 1999; 170: 105-111.
  • Marshall SE: Behçet’s disease. Best Practice & Research Clinical Rheumatology 2004;18: 291-311.
  • Demirhindi O, Yazıcı H, Binyıldız P, ve ark. Silivri fener köyü ve yöresinde Behçet hastalığı sıklığı ve bu hastalığın toplum içinde taranmasında kullanılabilecek bir yöntem. Cerrahp Tıp Fak Derg. 1981;12:509-514.
  • Yurdakul S, Yazici H, Tuzun Y, ve ark. THA arthritis of Behcet’s disease: a prospective study. Ann Rheum Dis 1983; 42: 505-515.
  • Gul A, Inanc M, Ocal L: Familial aggregation of Behcet’s disease in Turkey. Ann Rheum Dis 2000; 59:622-625.
  • Yazici H, Fresko I, Tunc R: Behcet’s syndrome: pathogenesis, clinical manifestations and treatment in Vasculitis by Gene V. Ball, S. Louis Bridges ed. Oxford University Press, USA; 2002;1:406-32.
  • Tüzün Y, Yurdakul S, Mat C, Özyazgan Y, Hamuryudan V, Tüzün B, Yazıcı H: Epidemiology of Behçet’s Syndrome in Turkey. International Journal of Dermatology 1996; 35: 618-620.
  • Gul A, Esin S, Dilsen N: Immunohistology of skin pathergy reaction in Behçet’s disease. British Journal of Dermatology 1995; 132: 901-907.
  • Demirkesen C, Tuzuner N, Mat C: Clinicopathologic evaluation of nodular cutaneous lesions of Behcet syndrome. Am J Clin Pathol 2001; 116: 341-346.
  • Saricaoglu H, Karadogan SK, Bayazit N, Yucel A, Dilek K, Tunali S. Clinical features of late onset Behcet’s disease: report of nine cases. Int J Dermatol 2006;45:1284-1287.
  • Calamia KT, Wilson FC, Icen M, Crowson CS, Gabriel SE, Kremers HM: Epidemiology and clinical characteristics of Behçet’s disease in the US: a population based study.Arthritis Rheum. 2009;61:600-604.
  • Yaylalı GF: Denizli ve Çevresinde Ailesel Akdeniz Ateşi ve Behçet Hastalığı Prevalansı: “Sıfır Hasta” Modelinin uygulanması. Uzmanlık tezi. Denizli, 2003.
  • Ando K, Fujino Y, Hijikata K: Epidemiological features and visual prognosis of Behçet’s disease. Jpn J Ophthalmol 1999;43: 312-317.
  • Bayraktar Y, Balkanci F, Bayraktar M: Budd-Chiari syndrome: a common complication of Behcet’s disease. Am J Gastroenterol 1997; 92: 858-86.
  • Benezra D, Cohen E: Treatment and visual prognosis in Behçet’s disease. Br J Ophthalmol 1986;70: 589-592.
  • Sakamoto M, Akazawa K, Nishioka Y: Prognostic factors of vision in patients with Behcet disease. Ophthalmology 1995;102:317-21.
  • Kural-Seyahi E, Fresko I, Seyahi N: The long-term mortality and morbidity of Behcet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore). 2003; 82:60-76.
  • Karaca M :Ailevi Behçet Hastalığı Olgularında Hedef Organ İlişkilerinin Faktör Analizi İle İncelenmesi. Uzmanlık Tezi, İstanbul, 2008.
  • Zouboulis C.C: Epidemiology of Adamantiades-Behcet’s disease. Ann Med Interne 1999; 150: 488-498.
  • Gurler A, Boyvat A, Tursen U: Clinical manifestations of Behcet’s disease: an analysis of 2147 patients. Yonsei Med J 1997; 38: 423-427.
  • Yazici H, Fresko I: Behcet’s disease and other autoinflammatory conditions: what’s in a name? Clin Exp Rheumatol 2005; 23 : 1-2.
  • Ilknur T, Pabuccuoglu U, Akin C, Lebe B, Gunes AT. Histopathologic and direct immunofluorescence findings of the papulopustular lesions in Behcet’s disease. Eur J Dermatol 2006;16:146-150.
  • Chang HK, Kim JU, Cheon KS, Chung HR , Lee KW, Lee IH: HLA-B51 and its allelic types in association with Behçet’s disease and recurrent aphthous stomatitis in Korea. Clin Exp Rheumatol. 2001 ; 19: 31-35.
  • Yıldırım M, Kılınç Y, Ceyhan MA. Behçet hastalığı patogenezindeki yenilikler S.D.Ü.Tıp Fak.Derg. 2009;16:29-34.
  • Kılınç Y: Behçet Hastalarında Yaşam Kalitesi Anksiyete ve Depresyon, Uzmanlık Tezi, Isparta, 2007.
  • Aslan SH, Soylu MB, Alparslan ZN, Ünal M: Behçet Hastalığında psikososyal etkenler ve ruhsal bulgular. Türk Psikiyatri Dergisi 1996;1:215-221.
  • Yazici H, Chamberlain MA, Schreuder I: HLA antigens in Behcet’s disease: a reappraisal by a comparative study of Turkish and British patients. Ann Rheum Dis. 1980 ;39:344-348.
  • Ohno S, Asanuma T, Sugiura S: HLA-Bw51 and Behcet’s disease. JAMA 1978; 240: 529.
  • Celenk C, Aydin F, Unsal M: Pulmonary alterations in Behcet’s disease. Europan Journal of Radiology, 2009;70:317-319.
  • Yazici H, Chamberlain MA, Schreuder GM: HLA B5 and Behcet’s disease. Ann Rheum Dis. 1983;42:602-603.
  • Verity DH, Marr JE, Ohno S: Behcet’s disease, the Silk Road and HLA- B51: historical and geographical perspectives. Tissue Antigens. 1999; 54: 213-220.
  • Gul A, Uyar FA, Inanc M: Lack of association of HLA-B*51 with a severe disease course in Behcet’s disease. Rheumatology 2001; 40: 668-672.
  • Muftuoglu AU, Yazici H, Yurdakul S: Behcet’s disease: lack of correlation of clinical manifestations with HLA antigens. Tissue Antigens. 1981; 17: 226-230.
  • Güner İ, Özmen D, Bayındır O: Sitokinler: Cytokines. T Klin J Med Sci, 1997;17:65-74.
  • Houman H, Hamzaoui A, Ghorbal IB, Khanfir M, Feki M, Hamzaoui K: Abnormal expression of chemokine receptors in Behçet’s disease: relationship to intracellular Th1/Th2 cytokines and to clinical manifestations. Journal of Autoimmunity 2004;23: 267-273.
  • Turan B, Gallati H, Erdi H, Gürler A, Michel BA, Villiger PM: Systemic levels of the T cell regulatory cytokines IL-10 and IL-12 in Behçet’s disease; soluble TNFR-75 as a biological marker of disease activity. J Rheumatol. 1997 ; 24 : 128-132.
  • Akman-Demir G, Tüzün E, Içöz S, Yeşilot N, Yentür SP, Kürtüncü M, Mutlu M, Saruhan-Direskeneli G: Interleukin-6 in neuro-Behçet’s disease: association with disease subsets and long-term outcome. Cytokine, 2008; 44: 373-376.
  • Curnow SJ, Pryce K, Modi N, Knight B, Graham EM, Stewart JE, Fortune F, Stanford MR, Murray PI, Wallace GR: Serum cytokine profiles in Behçet’s disease: Is there a role for IL-15 in pathogenesis? Immunol Lett. 2008;121:7-12.
  • Hamuryudan V, Yurdakul S, Serdaroglu S, Tuzun Y, Rosenkaimer F, Yazici H. Topical alpha interferon in the treatment of oral ulcers in Behcet’s syndrome: a preliminary report. Clin Exp Rheumatol 1990;8:51-54.
  • Mochizuki M, Morita E, Yamamoto S, Yamana S: Characteristics of T cell lines established from skin lesions of Behcet’s disease. Journal of Dermatological Science 1997; 15: 9-13.
  • Hatemi G, Bahar H, Uysal S: The pustular skin lesions in Behcet’s syndrome are not sterile. Ann Rheum Dis 2004; 63: 1450-1452.
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There are 82 citations in total.

Details

Primary Language Turkish
Journal Section Articles
Authors

Emre Aytuğar This is me

Filiz Namdar Pekiner

Publication Date January 31, 2014
Submission Date November 1, 2013
Published in Issue Year 2011 Volume: 1 Issue: 1

Cite

APA Aytuğar, E., & Namdar Pekiner, F. (2014). Behçet hastalığı. Clinical and Experimental Health Sciences, 1(1), 65-73.
AMA Aytuğar E, Namdar Pekiner F. Behçet hastalığı. Clinical and Experimental Health Sciences. January 2014;1(1):65-73.
Chicago Aytuğar, Emre, and Filiz Namdar Pekiner. “Behçet hastalığı”. Clinical and Experimental Health Sciences 1, no. 1 (January 2014): 65-73.
EndNote Aytuğar E, Namdar Pekiner F (January 1, 2014) Behçet hastalığı. Clinical and Experimental Health Sciences 1 1 65–73.
IEEE E. Aytuğar and F. Namdar Pekiner, “Behçet hastalığı”, Clinical and Experimental Health Sciences, vol. 1, no. 1, pp. 65–73, 2014.
ISNAD Aytuğar, Emre - Namdar Pekiner, Filiz. “Behçet hastalığı”. Clinical and Experimental Health Sciences 1/1 (January 2014), 65-73.
JAMA Aytuğar E, Namdar Pekiner F. Behçet hastalığı. Clinical and Experimental Health Sciences. 2014;1:65–73.
MLA Aytuğar, Emre and Filiz Namdar Pekiner. “Behçet hastalığı”. Clinical and Experimental Health Sciences, vol. 1, no. 1, 2014, pp. 65-73.
Vancouver Aytuğar E, Namdar Pekiner F. Behçet hastalığı. Clinical and Experimental Health Sciences. 2014;1(1):65-73.

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