The Prognosis of Behçet Syndrome

  • Emire Seyahi
  • David Saadoun


Behçet’s syndrome (BS) may cause several serious morbidities and a fatal outcome. A 20-year follow-up study found an overall mortality rate of 5–10% and increased standardized mortality ratios among young males. Major causes of death were large vessel and parenchymal central nervous system involvement. Mortality rate tends to decrease with time. The same trend is observed in the severity of eye disease and the frequency of skin–mucosa lesions. However, parenchymal CNS disease and peripheral artery aneurysms may occur late during the disease course. Also, male patients with BS are more prone to develop serious morbidities at long term even when they have no major organ involvement during the early years of their disease. Major morbidity results from eye, vascular, and neurological involvement. The outcome of eye disease is improving significantly with biological agents. However, still about 10% of patients may become blind despite treatment. Similarly, prognosis is still grim for pulmonary artery aneurysms, which lead to death in about one fourth of the affected cases, and parenchymal neurological involvement, which results in death or severe disability in approximately half of the affected cases. Here we review several aspects of morbidity and mortality due to BS.


Behçet’s syndrome Prognosis Mortality Morbidity Eye disease Vascular involvement Neurological involvement Cancer 


  1. 1.
    Kural-Seyahi E, Fresko I, Seyahi N, et al. The long-term mortality and morbidity of Behçet’s syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore). 2003;82(1):60–76.CrossRefGoogle Scholar
  2. 2.
    Yazici H, Tüzün Y, Pazarli H, et al. Influence of age of onset and patient’s sex on the prevalence and severity of manifestations of Behçet’s syndrome. Ann Rheum Dis. 1984;43:783–9.PubMedPubMedCentralCrossRefGoogle Scholar
  3. 3.
    Chajek T, Fainaru M. Behçet’s disease. Report of 41 cases and a review of the literature. Medicine (Baltimore). 1975;54:179–96.CrossRefGoogle Scholar
  4. 4.
    Mishima Y, Ishikawa K, Ueno A. Arterial involvement in Behçet’s disease. Jpn J Surg. 1973;3:52–60.PubMedCrossRefGoogle Scholar
  5. 5.
    Shimizu T, Ehrlich GE, Inaba G, Hayashi K. Behçet’s disease (Behçet’s syndrome). Semin Arthritis Rheum. 1979;8:223–60.PubMedCrossRefGoogle Scholar
  6. 6.
    Yazici H, Başaran G, Hamuryudan V, et al. The ten-year mortality in Behçet’s syndrome. Br J Rheumatol. 1996;35:139–41.PubMedCrossRefGoogle Scholar
  7. 7.
    Kaklamani VG, Vaiopoulos G, Kaklamanis PG. Behçet’s disease. Semin Arthritis Rheum. 1998;27:197–217.PubMedCrossRefGoogle Scholar
  8. 8.
    Benamour S, Zeroual B, Bennis R, Amraoui A Bettal S [Behçet’s disease. 316 cases]. Presse Med. 1990;19:1485–9.Google Scholar
  9. 9.
    Yamamoto S, Toyokawa H, Matsubara J, et al. A nation-wide survey of Behçet’s disease in Japan, 1. Epidemiological survey. Jpn J Ophthalmol. 1974;18:282–90.Google Scholar
  10. 10.
    Park KD, Bang D, Lee ES, Lee SH, Lee S. Clinical study on death in Behçet’s disease. J Korean Med Sci. 1993;8:241–5.PubMedPubMedCentralCrossRefGoogle Scholar
  11. 11.
    Saadoun D, Wechsler B, Desseaux K, et al. Mortality in Behçet’s disease. Arthritis Rheum. 2010;62:2806–12.PubMedPubMedCentralCrossRefGoogle Scholar
  12. 12.
    Savey L, Resche-Rigon M, Wechsler B, et al. Ethnicity and association with disease manifestations and mortality in Behçet’s disease. Orphanet J Rare Dis. 2014;9:42.PubMedPubMedCentralCrossRefGoogle Scholar
  13. 13.
    Urowitz MB, Bookman AA, Koehler BE, Gordon DA, Smythe HA, Ogryzlo MA. The bimodal mortality pattern of systemic lupus erythematosus. Am J Med. 1976;60:221–5.PubMedCrossRefGoogle Scholar
  14. 14.
    Wolfe F, Mitchell DM, Sibley JT, et al. The mortality of rheumatoid arthritis. Arthritis Rheum. 1994;37:481–94.PubMedCrossRefGoogle Scholar
  15. 15.
    Manzi S, Meilahn EN, Rairie JE, et al. Age-specific incidence rates of myocardial infarction and angina in women with systemic lupus erythematosus: comparison with the Framingham study. Am J Epidemiol. 1997;145:408–15.PubMedCrossRefGoogle Scholar
  16. 16.
    Chung CP, Oeser A, Raggi P, et al. Increased coronary-artery atherosclerosis in rheumatoid arthritis: relationship to disease duration and cardiovascular risk factors. Arthritis Rheum. 2005;52:3045–53.PubMedCrossRefGoogle Scholar
  17. 17.
    Lee YB, Lee SY, Choi JY, et al. Incidence, prevalence, and mortality of Adamantiades-Behçet’s disease in Korea: a nationwide, population-based study (2006-2015). J Eur Acad Dermatol Venereol. 2018;32:999–1003.PubMedCrossRefGoogle Scholar
  18. 18.
    Mansur AT, Kocaayan N, Serdar ZA, Alptekin F. Giant oral ulcers of Behçet’s disease mimicking squamous cell carcinoma. Acta Derm Venereol. 2005;85:532–4.PubMedGoogle Scholar
  19. 19.
    Almoznino G, Ben-Chetrit E. Infliximab for the treatment of resistant oral ulcers in Behçet’s disease: a case report and review of the literature. Clin Exp Rheumatol. 2007;25(4 Suppl 45):S99–S102.PubMedGoogle Scholar
  20. 20.
    Tugal-Tutkun I, Onal S, Altan-Yaycioglu R, Huseyin Altunbas H, Urgancioglu M. Uveitis in Behçet’s disease: an analysis of 880 patients. Am J Ophthalmol. 2004;138:373–80.PubMedPubMedCentralCrossRefGoogle Scholar
  21. 21.
    Cingu AK, Onal S, Urgancioglu M, Tugal-Tutkun I. Comparison of presenting features and three-year disease course in Turkish patients with Behçet uveitis who presented in the early 1990s and the early 2000s. Ocul Immunol Inflamm. 2012;20:423–8.PubMedCrossRefGoogle Scholar
  22. 22.
    Sakamoto M, Akazawa K, Nishioka Y, Sanui H, Inomata H, Nose Y. Prognostic factors of vision in patients with Behçet’s disease. Ophthalmology. 1995;102:317–21.PubMedCrossRefGoogle Scholar
  23. 23.
    Takeuchi M, Hokama H, Tsukahara R, Kezuka T, Goto H, Sakai J, Usui M. Risk and prognostic factors of poor visual outcome in Behçet’s disease with ocular involvement. Graefes Arch Clin Exp Ophthalmol. 2005;243:1147–52.PubMedCrossRefGoogle Scholar
  24. 24.
    Mamo JG, Baghdassarian A. Behçet’s disease. Arch Ophthalmol. 1964;71:38–48.CrossRefGoogle Scholar
  25. 25.
    Mishima S, Masuda K, Izawa Y, Mochizuke M, Namba K. Behçet’s disease in Japan: ophthalmological aspects. Tr Am Ophthalmol Soc. 1979;77:225–79.Google Scholar
  26. 26.
    Benezra D, Cohen E. Treatment and visual prognosis in Behçet’s disease. Br J Ophthalmol. 1986;70:589–92.PubMedPubMedCentralCrossRefGoogle Scholar
  27. 27.
    Yazici H, Pazarli H, Barnes CG, et al. A controlled trial of azathioprine in Behçet’s syndrome. N Engl J Med. 1990;322:281–5.PubMedPubMedCentralCrossRefGoogle Scholar
  28. 28.
    Hamuryudan V, Ozyazgan Y, Hizli N, et al. Azathioprine in Behçet’s syndrome: effects on long-term prognosis. Arthritis Rheum. 1997;40:769–74.PubMedCrossRefGoogle Scholar
  29. 29.
    Cho YJ, Kim WK, Lee JH, et al. Visual prognosis and risk factors for Korean patients with Behçet’s uveitis. Ophthalmologica. 2008;222:344–50.PubMedCrossRefGoogle Scholar
  30. 30.
    Yoshida A, Kawashima H, Motoyama Y, et al. Comparison of patients with Behçet’s disease in the 1980s and 1990s. Ophthalmology. 2004;111:810–5.PubMedPubMedCentralCrossRefGoogle Scholar
  31. 31.
    Ando K, Fujino Y, Hijikata K, Izawa Y, Masuda K. Epidemiological features and visual prognosis of Behçet’s disease. Jpn J Ophthalmol. 1999;43:312–7.PubMedCrossRefGoogle Scholar
  32. 32.
    Chung YM, Lin YC, Tsai CC, Huang DF. Behçet’s disease with uveitis in Taiwan. J Chin Med Assoc. 2008;71:509–16.PubMedCrossRefGoogle Scholar
  33. 33.
    Kump LI, Moeller KL, Reed GF, Kurup SK, Nussenblatt RB, Levy-Clarke GA. Behçet’s disease: comparing 3 decades of treatment response at the National Eye Institute. Can J Ophthalmol. 2008;43:468–72.PubMedPubMedCentralCrossRefGoogle Scholar
  34. 34.
    Taylor SR, Singh J, Menezo V, Wakefield D, McCluskey P, Lightman S. Behçet disease: visual prognosis and factors influencing the development of visual loss. Am J Ophthalmol. 2011;152:1059–66.PubMedCrossRefGoogle Scholar
  35. 35.
    Accorinti M, Pesci FR, Pirraglia MP, Abicca I, Pivetti-Pezzi P. Ocular Behçet’s disease: changing patterns over time, complications and long-term visual prognosis. Ocul Immunol Inflamm. 2017;25(1):29–36.PubMedCrossRefGoogle Scholar
  36. 36.
    Arevalo JF, Lasave AF, Al Jindan MY, Al Sabaani NA, Al-Mahmood AM, Al-Zahrani YA, Al Dhibi HA, KKESH Uveitis Survey Study Group, KKESH Uveitis Survey Study Group. Uveitis in Behçet disease in a tertiary center over 25 years: the KKESH Uveitis Survey Study Group. Am J Ophthalmol. 2015;159(1):177–84.e1–2.PubMedCrossRefGoogle Scholar
  37. 37.
    Chung YR, Lee ES, Kim MH, Lew HM, Song JH. Changes in ocular manifestations of Behçet disease in Korean patients over time: a single-center experience in the 1990s and 2000s. Ocul Immunol Inflamm. 2015;23(2):157–61.PubMedCrossRefGoogle Scholar
  38. 38.
    Amer R, Alsughayyar W, Almeida D. Pattern and causes of visual loss in Behçet’s uveitis: short-term and long-term outcomes. Graefes Arch Clin Exp Ophthalmol. 2017;255:1423–32.PubMedCrossRefGoogle Scholar
  39. 39.
    Kaçmaz RO, Kempen JH, Newcomb C, Gangaputra S, Daniel E, Levy-Clarke GA, et al. Ocular inflammation in Behçet disease: incidence of ocular complications and of loss of visual acuity. Am J Ophthalmol. 2008;146(6):828–36.PubMedPubMedCentralCrossRefGoogle Scholar
  40. 40.
    Tugal-Tutkun I, Mudun A, Urgancioglu M, et al. Efficacy of infliximab in the treatment of uveitis that is resistant to treatment with the combination of azathioprine, cyclosporine, and corticosteroids in Behçet’s disease: an open-label trial. Arthr Rheum. 2005;52:2478–84.CrossRefGoogle Scholar
  41. 41.
    Diwo E, Gueudry J, Saadoun D, Weschler B, LeHoang P, Bodaghi B. Long-term efficacy of interferon in severe uveitis associated with Behçet disease. Ocul Immunol Inflamm. 2017;25(1):76–84.PubMedCrossRefGoogle Scholar
  42. 42.
    Hamza M. Large artery involvement in Behçet’s disease. J Rheumatol. 1987;14:554–9.PubMedGoogle Scholar
  43. 43.
    Koc Y, Gullu I, Akpek G, et al. Vascular involvement in Behçet’s disease. J Rheumatol. 1992;19:402–10.PubMedGoogle Scholar
  44. 44.
    Hamuryudan V, Yurdakul S, Moral F, et al. Pulmonary arterial aneurysms in Behçet’s syndrome: a report of 24 cases. Br J Rheumatol. 1994;33:48–51.PubMedCrossRefGoogle Scholar
  45. 45.
    Tüzün H, Besirli K, Sayin A, Yazici H, et al. Management of aneurysms in Behçet’s syndrome: an analysis of 24 patients. Surgery. 1997;121:150–6.PubMedPubMedCentralCrossRefGoogle Scholar
  46. 46.
    Bayraktar Y, Balkanci F, Bayraktar M, Calguneri M. Budd–Chiari syndrome: a common complication of Behçet’s disease. Am J Gastroenterol. 1997;92:858–62.PubMedGoogle Scholar
  47. 47.
    Hamuryudan V, Er T, Seyahi E, et al. Pulmonary artery aneurysms in Behçet’s syndrome. Am J Med. 2004;117:867–70.PubMedCrossRefGoogle Scholar
  48. 48.
    Düzgun N, Ateş A, Aydintuğ OT, Demir O, Olmez U. Characteristics of vascular involvement in Behçet’s disease. Scand J Rheumatol. 2006;35:65–8.PubMedCrossRefGoogle Scholar
  49. 49.
    Seyahi E, Melikoglu M, Akman C, Hamuryudan V, Ozer H, Hatemi G, et al. Pulmonary artery involvement and associated lung disease in Behçet disease: a series of 47 patients. Medicine (Baltimore). 2012;91(1):35–48.CrossRefGoogle Scholar
  50. 50.
    Desbois AC, Rautou PE, Biard L, Belmatoug N, Wechsler B, Resche-Rigon M, et al. Behcet’s disease in Budd-Chiari syndrome. Orphanet J Rare Dis. 2014;9:104.PubMedPubMedCentralCrossRefGoogle Scholar
  51. 51.
    Uluduz D, Kürtüncü M, Yapıcı Z, Seyahi E, Kasapçopur Ö, Özdoğan H, et al. Clinical characteristics of pediatric-onset neuro-Behçet disease. Neurology. 2011;77(21):1900–5.CrossRefGoogle Scholar
  52. 52.
    Tuzun H, Seyahi E, Arslan C, Hamuryudan V, Besirli K, Yazici H. Management and prognosis of nonpulmonary large arterial disease in patients with Behçet disease. J Vasc Surg. 2012;55(1):157–63.PubMedCrossRefGoogle Scholar
  53. 53.
    Saadoun D, Asli B, Wechsler B, Houman H, Geri G, Desseaux K, et al. Long-term outcome of arterial lesions in Behçet disease: a series of 101 patients. Medicine (Baltimore). 2012;91(1):18–24.CrossRefGoogle Scholar
  54. 54.
    Geri G, Wechsler B, Thi Huong d L, Isnard R, Piette JC, Amoura Z, et al. Spectrum of cardiac lesions in Behçet disease: a series of 52 patients and review of the literature. Medicine (Baltimore). 2012;91(1):25–34.CrossRefGoogle Scholar
  55. 55.
    Desbois AC, Wechsler B, Resche-Rigon M, Piette JC, Huong Dle T, Amoura Z, et al. Immunosuppressants reduce venous thrombosis relapse in Behçet’s disease. Arthritis Rheum. 2012;64(8):2753–60.PubMedCrossRefGoogle Scholar
  56. 56.
    Tascilar K, Melikoglu M, Ugurlu S, Sut N, Caglar E, Yazici H. Vascular involvement in Behçet’s syndrome: a retrospective analysis of associations and the time course. Rheumatology (Oxford). 2014;53(11):2018–22.CrossRefGoogle Scholar
  57. 57.
    Seyahi E, Caglar E, Ugurlu S, Kantarci F, Hamuryudan V, Sonsuz A, et al. An outcome survey of 43 patients with Budd-Chiari syndrome due to Behçet’s syndrome followed up at a single, dedicated center. Semin Arthritis Rheum. 2015;44(5):602–9.PubMedCrossRefPubMedCentralGoogle Scholar
  58. 58.
    Cansu DU, Temel T, Erturk A, Kasifoglu T, Acu B, Korkmaz C. The long-term outcomes for patients with Budd-Chiari syndrome caused by Behcet’s disease: a case series on the results, from cirrhosis to death. Hepat Mon. 2016;16(10):e32457.PubMedPubMedCentralCrossRefGoogle Scholar
  59. 59.
    Akman-Demir G, Serdaroglu P, Tasci B. Clinical patterns of neurological involvement in Behçet’s disease: evaluation of 200 patients. The Neuro-Behçet’s Study Group. Brain. 1999;122:2171–82.CrossRefGoogle Scholar
  60. 60.
    Siva A, Kantarci OH, Saip S, Altintas A, Hamuryudan V, Islak C, et al. Behçet’s disease: diagnostic and prognostic aspects of neurological involvement. J Neurol. 2001;248:95–103.PubMedPubMedCentralCrossRefGoogle Scholar
  61. 61.
    Houman MH, Bellakhal S, Ben Salem T, Hamzaoui A, Braham A, Lamloum M, et al. Characteristics of neurological manifestations of Behçet’s disease: a retrospective monocentric study in Tunisia. Clin Neurol Neurosurg. 2013;115(10):2015–8.PubMedCrossRefGoogle Scholar
  62. 62.
    Ideguchi H, Suda A, Takeno M, Kirino Y, Ihata A, Ueda A, et al. Neurological manifestations of Behçet’s disease in Japan: a study of 54 patients. J Neurol. 2010;257(6):1012–20.PubMedCrossRefGoogle Scholar
  63. 63.
    Talarico R, d’Ascanio A, Figus M, Stagnaro C, Ferrari C, Elefante E, et al. Behçet’s disease: features of neurological involvement in a dedicated centre in Italy. Clin Exp Rheumatol. 2012;30(3 Suppl 72):S69–72.PubMedGoogle Scholar
  64. 64.
    Akman-Demir G, Tüzün E, Içöz S, Yeşilot N, Yentür SP, Kürtüncü M, et al. Interleukin-6 in neuro-Behçet’s disease: association with disease subsets and long-term outcome. Cytokine. 2008;44(3):373–6.PubMedCrossRefGoogle Scholar
  65. 65.
    Yesilot N, Mutlu M, Gungor O, Baykal B, Serdaroglu P, Akman-Demir G. Clinical characteristics and course of spinal cord involvement in Behçet’s disease. Eur J Neurol. 2007;14(7):729–37.PubMedCrossRefGoogle Scholar
  66. 66.
    Hirohata S, Kikuchi H, Sawada T, Nagafuchi H, Kuwana M, Takeno M, et al. Retrospective analysis of long-term outcome of chronic progressive neurological manifestations in Behcet’s disease. J Neurol Sci. 2015;349(1–2):143–8.PubMedCrossRefGoogle Scholar
  67. 67.
    Noel N, Bernard R, Wechsler B, Resche-Rigon M, Depaz R, Le Thi Huong Boutin D, et al. Long-term outcome of neuro-Behçet’s disease. Arthritis Rheumatol. 2014;66(5):1306–14.PubMedCrossRefGoogle Scholar
  68. 68.
    Tunc R, Saip S, Siva A, Yazici H. Cerebral venous thrombosis is associated with major vessel disease in Behçet’s syndrome. Ann Rheum Dis. 2004;63:1693–4.PubMedPubMedCentralCrossRefGoogle Scholar
  69. 69.
    Saadoun D, Wechsler B, Resche-Rigon M, Trad S, Le Thi Huong D, Sbai A, et al. Cerebral venous thrombosis in Behçet’s disease. Arthritis Rheum. 2009;61(4):518–26.PubMedCrossRefGoogle Scholar
  70. 70.
    Aguiar de Sousa D, Mestre T, Ferro JM. Cerebral venous thrombosis in Behçet’s disease: a systematic review. J Neurol. 2011;258(5):719–27.PubMedCrossRefGoogle Scholar
  71. 71.
    Uluduz D, Midi I, Duman T, Colakoglu S, Tüfekci A, Bakar M, et al. Behçet’s disease as a causative factor of cerebral venous sinus thrombosis: subgroup analysis of data from the VENOST study. Rheumatology (Oxford). 2018;58(4):600–8.CrossRefGoogle Scholar
  72. 72.
    Cheon JH, Kim WH. An update on the diagnosis, treatment, and prognosis of intestinal Behçet’s disease. Curr Opin Rheumatol. 2015;27:24–31.PubMedCrossRefGoogle Scholar
  73. 73.
    Skef W, Hamilton MJ, Arayssi T. Gastrointestinal Behçet’s disease: a review. World J Gastroenterol. 2015;21(13):3801–12.PubMedPubMedCentralCrossRefGoogle Scholar
  74. 74.
    Jung YS, Cheon JH, Park SJ, et al. Clinical course of intestinal Behcet’s disease during the first five years. Dig Dis Sci. 2013;58:496–503.PubMedCrossRefGoogle Scholar
  75. 75.
    Hatemi I, Esatoglu SN, Hatemi G, Erzin Y, Yazici H, Celik AF. Characteristics, treatment, and long-term outcome of gastrointestinal involvement in Behcet’s syndrome: a strobe-compliant observational study from a dedicated multidisciplinary center. Medicine (Baltimore). 2016;95(16):e3348.CrossRefGoogle Scholar
  76. 76.
    Choi IJ, Kim JS, Cha SD, Jung HC, Park JG, Song IS, Kim CY. Long-term clinical course and prognostic factors in intestinal Behçet’s disease. Dis Colon Rectum. 2000;43:692–700.CrossRefGoogle Scholar
  77. 77.
    Chung MJ, Cheon JH, Kim SU, Park JJ, Kim TI, Kim NK, Kim WH. Response rates to medical treatments and long-term clinical outcomes of nonsurgical patients with intestinal Behçet disease. J Clin Gastroenterol. 2010;44:e116–22.PubMedGoogle Scholar
  78. 78.
    Jung YS, Cheon JH, Park SJ, Hong SP, Kim TI, Kim WH. Long-term clinical outcomes of Crohn’s disease and intestinal Behcet’s disease. Inflamm Bowel Dis. 2013;19(1):99–105.PubMedCrossRefGoogle Scholar
  79. 79.
    Kim JS, Lim SH, Choi IJ, Moon H, Jung HC, Song IS, Kim CY. Prediction of the clinical course of Behçet’s colitis according to macroscopic classification by colonoscopy. Endoscopy. 2000;32:635–40.PubMedCrossRefPubMedCentralGoogle Scholar
  80. 80.
    Hamuryudan V, Hatemi G, Tascilar K, Sut N, Ozyazgan Y, Seyahi E, et al. Prognosis of Behcet’s syndrome among men with mucocutaneous involvement at disease onset: long-term outcome of patients enrolled in a controlled trial. Rheumatology (Oxford). 2010;49(1):173.CrossRefGoogle Scholar
  81. 81.
    Hamuryudan V, Mat C, Saip S, Ozyazgan Y, Siva A, Yurdakul S, et al. Thalidomide in the treatment of the mucocutaneous lesions of the Behçet syndrome. A randomized, double-blind, placebo-controlled trial. Ann Intern Med. 1998;128(6):443–50.PubMedCrossRefGoogle Scholar
  82. 82.
    Talarico R, Cantarini L, d’Ascanio A, Figus M, Favati B, Baldini C, et al. Development of de novo major involvement during follow-up in Behçet’s syndrome. Clin Rheumatol. 2016;35(1):247–50.PubMedCrossRefGoogle Scholar
  83. 83.
    Alpsoy E, Donmez L, Onder M, et al. Clinical features and natural course of Behçet’s disease in 661 cases: a multicentre study. Br J Dermatol. 2007;157:901–6.CrossRefGoogle Scholar
  84. 84.
    Fidaner C, Eser SY, Parkin DM. Incidence in Izmir in 1993–1994: first results from Izmir Cancer Registry. Eur J Cancer. 2001;37:83–92.PubMedCrossRefGoogle Scholar
  85. 85.
    Kaklamani VG, Tzonou A, Kaklamanis PG. Behçet’s disease associated with malignancies. Report of two cases and review of the literature. Clin Exp Rheumatol. 2005;23(4 Suppl 38):S35–41.PubMedGoogle Scholar
  86. 86.
    Wang LH, Wang WM, Hsu SM, Lin SH, Shieh CC. Risk of overall and site-specific cancers in Behçet disease: a Nationwide Population-based Study in Taiwan. J Rheumatol. 2015;42(5):879–84.PubMedCrossRefPubMedCentralGoogle Scholar
  87. 87.
    Jung YS, Han M, Kim DY, Cheon JH, Park S. Cancer risk in Korean patients with Behçet’s disease: a nationwide population-based study. PLoS One. 2017;12(12):e0190182.PubMedPubMedCentralCrossRefGoogle Scholar
  88. 88.
    Na SJ, Kang MJ, Yu DS, et al. Cancer risk in patients with Behçet disease: a nationwide population-based dynamic cohort study from Korea. J Am Acad Dermatol. 2017;78:464–70.e2.PubMedCrossRefGoogle Scholar
  89. 89.
    Lin Y, Li G, Zheng W, Tian X, Zhang F. Behcet’s disease associated with malignancy: a report of 41 Chinese cases. Int J Rheum Dis. 2014;17(4):459–65.PubMedCrossRefGoogle Scholar
  90. 90.
    Ahn JK, Oh JM, Lee J, Koh EM, Cha HS. Behcet’s disease associated with malignancy in Korea: a single center experience. Rheumatol Int. 2010;30(6):831–5.PubMedCrossRefGoogle Scholar
  91. 91.
    Lin YC, Liang TH, Chang HN, Lin JS, Lin HY. Behçet disease associated with myelodysplastic syndrome. J Clin Rheumatol. 2008;14(3):169–74.PubMedCrossRefGoogle Scholar
  92. 92.
    Esatoglu SN, Hatemi G, Salihoglu A, Hatemi I, Soysal T, Celik AF. A reappraisal of the association between Behçet’s disease, myelodysplastic syndrome and the presence of trisomy 8: a systematic literature review. Clin Exp Rheumatol. 2015;33(6 Suppl 94):S145–51.PubMedPubMedCentralGoogle Scholar
  93. 93.
    Kanamitsu K, Shimada A, Nishiuchi R, Shigemura T, Nakazawa Y, Koike K, et al. Pediatric intestinal Behçet disease complicated by myeloid malignancies. Int J Hematol. 2017;105(3):377–82.PubMedCrossRefGoogle Scholar
  94. 94.
    Han M, Jung YS, Kim WH, Cheon JH, Park S. Cancer risk in patients with intestinal Behçet’s disease: a Nationwide population-based study. Gut Liver. 2018;12(4):433–9.PubMedPubMedCentralCrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Emire Seyahi
    • 1
  • David Saadoun
    • 2
  1. 1.Division of Rheumatology, Department of Internal MedicineIstanbul University-CerrahpasaIstanbulTurkey
  2. 2.Department of Internal Medicine and Clinical ImmunologyCentre national de référence des maladies Autoimmunes Systémiques rares, Centre national de référence des maladies Autoinflammatoires et de l’Amylose; Groupe Hospitalier Pitié-Salpêtrière, AP-HP; Sorbonne Universités, UPMC Univ Paris 06ParisFrance

Personalised recommendations