Skip to main content
SpringerLink
Log in

Arterial and Venous Involvement in Behçet’s Disease

  • Chapter
  • First Online:
Large and Medium Size Vessel and Single Organ Vasculitis

Part of the book series: Rare Diseases of the Immune System ((RDIS))

  • 780 Accesses

Abstract

Behçet’s disease (BD) is a chronic, multisystemic, inflammatory disease characterized by recurrent attacks of mucocutaneous, ocular, musculoskeletal, vascular, central nervous system and gastrointestinal manifestations. Vascular involvement is observed in up to 40% of the patients with BD, especially in young males and is one of the major causes of mortality and morbidity. Both venous and arterial disease is observed. Glucocorticoids, azathioprine and cyclophosphamide are recommended as the first-line treatments in vascular BD (VBD). But increasing data with TNF inhibitors and interferons suggest that these agents may also be acceptable options for the management of refractory cases. Anticoagulant usage is still controversial with limited data coming from retrospective studies. There is a clear need for randomized, controlled studies for the management of VBD.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
eBook
USD 16.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 159.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Calamia KT, Schirmer M, Melikoglu M. Major vessel involvement in Behcçet’s disease: an update. Curr Opin Rheumatol. 2011;23:24–31.

    Article  PubMed  Google Scholar 

  2. Yazici H, Yurdakul S, Hamuryudan V. Behcçet disease. Curr Opin Rheumatol. 2001;13:18–22.

    Article  CAS  PubMed  Google Scholar 

  3. Ozguler Y, Esatoglu SN, Seyahi E, Melikoglu M. Vascular and cardiac involvement. In: Yazici Y, et al., editors. Behçet syndrome. 2nd ed. Springer Nature Switzerland AG; 2020. p. 83–103.

    Google Scholar 

  4. Ishido T, Horita N, Takeuchi M, Kawagoe T, Shibuya E, Yamane T, et al. Clinical manifestations of Behçet’s disease depending on sex and age: results from Japanese Nationwide Registration. Rheumatology (Oxford). 2017;56(11):1918–27.

    Article  Google Scholar 

  5. Ryu HJ, Seo MR, Choi HJ, Baek HJ. Clinical phenotypes of Korean patients with Behcet disease according to gender, age at onset, and HLA-B51. Korean J Intern Med. 2018;33(5):1025–31.

    Article  CAS  PubMed  Google Scholar 

  6. Tascilar K, Melikoglu M, Ugurlu S, Sut N, Caglar E, Yazici H. Vascular involvement in Behcet’s syndrome: a retrospective analysis of associations and the time course. Rheumatology (Oxford). 2014;53(11):2018–22.

    Article  CAS  Google Scholar 

  7. Alibaz-Oner F, Karadeniz A, Yılmaz S, Balkarlı A, Kimyon G, Yazıcı A. Behçet disease with vascular involvement: effects of different therapeutic regimens on the incidence of new relapses. Medicine (Baltimore). 2015;94(6):e494.

    Article  CAS  Google Scholar 

  8. Seyahi E. Behcet’s disease: how to diagnose and treat vascular involvement. Best Pract Res Clin Rheumatol. 2016;30(2):279–95.

    Article  PubMed  Google Scholar 

  9. Saadoun D, Wechsler B, Desseaux K, Le Thi Huong D, Amoura Z, Resche-Rigon M, et al. Mortality in Behçet’s disease. Arthritis Rheum. 2010;62:2806–12.

    Article  CAS  PubMed  Google Scholar 

  10. Kural-Seyahi E, Fresko I, Seyahi N, Ozyazgan Y, Mat C, Hamuryudan V, et al. 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.

    Article  Google Scholar 

  11. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised international chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65:1–11.

    Article  CAS  PubMed  Google Scholar 

  12. Yazici H, Seyahi E, Hatemi G, Yazici Y. Behcet syndrome: a contemporary view. Nat Rev Rheumatol. 2018;14(2):119.

    Article  PubMed  Google Scholar 

  13. Yazawa S, Ishihara A, Kawasaki S. Fatal thoracic aortic aneurysm in a patient with childhood-onset vasculo-Behçet’s disease: an autopsy report. Intern Med. 2001;40(11):1154–7.

    Article  CAS  PubMed  Google Scholar 

  14. Fukuda Y, Watanabe I, Hayashi H, Kuwabara N. Pathological studies on Behçet’s disease. Ryumachi. 1980;20(4):268–75.

    CAS  PubMed  Google Scholar 

  15. Lakhanpal S, Tani K, Lie JT, Katoh K, Ishigatsubo Y, Ohokubo T. Pathologic features of Behçet’s syndrome: a review of Japanese autopsy registry data. Hum Pathol. 1985;16(8):790–5.

    Article  CAS  PubMed  Google Scholar 

  16. Ko GY, Byun JY, Choi BG, Cho SH. The vascular manifestations of Behçet’s disease: angiographic and CT findings. Br J Radiol. 2000;73(876):1270–4.

    Article  CAS  PubMed  Google Scholar 

  17. Mogulkoc N, Burgess MI, Bishop PW. Intracardiac thrombus in Behcet’s disease: a systematic review. Chest. 2000;118(2):479–87.

    Article  CAS  PubMed  Google Scholar 

  18. Kobayashi M, Ito M, Nakagawa A, Matsushita M, Nishikimi N, Sakurai T, et al. Neutrophil and endothelial cell activation in the vasa vasorum in vasculo-Behcet disease. Histopathology. 2000;36(4):362–71.

    Article  CAS  PubMed  Google Scholar 

  19. Matsumoto T, Uekusa T, Fukuda Y. Vasculo-Behcet’s disease: a pathologic study of eight cases. Hum Pathol. 1991;22(1):45–51.

    Article  CAS  PubMed  Google Scholar 

  20. Kim B, LeBoit PE. Histopathologic features of erythema nodosum—like lesions in Behçet disease: a comparison with erythema nodosum focusing on the role of vasculitis. Am J Dermatopathol. 2000;22(5):379–90.

    Article  CAS  PubMed  Google Scholar 

  21. Kiraz S, Ertenli I, Ozturk MA, Haznedaroglu IC, Celik I, Calguneri M. Pathological haemostasis and “prothrombotic state” in Behcet’s disease. Thromb Res. 2002;105(2):125–33.

    Article  CAS  PubMed  Google Scholar 

  22. Chamorro AJ, Marcos M, Hernandez-Garcia I, Calvo A, Mejia JC, Cervera R, et al. Association of allelic variants of factor V Leiden, prothrombin and methylenetetrahydrofolate reductase with thrombosis or ocular involvement in Behcet’s disease: a systematic review and meta-analysis. Autoimmun Rev. 2013;12(5):607–16.

    Article  CAS  PubMed  Google Scholar 

  23. La Regina M, Orlandini F, Prisco D, Dentali F. Homocysteine in vascular Behcet disease: a meta-analysis. Arterioscler Thromb Vasc Biol. 2010;30(10):2067–74.

    Article  PubMed  Google Scholar 

  24. Tokay S, Direskeneli H, Yurdakul S, Akoglu T. Anticardiolipin antibodies in Behçet’s disease: a reassessment. Rheumatology (Oxford). 2001;40(2):192–5.

    Article  CAS  Google Scholar 

  25. Yaşar NŞ, Salgür F, Cansu DÜ, Kaşifoğlu T, Korkmaz C. Combined thrombophilic factors increase the risk of recurrent thrombotic events in Behcet’s disease. Clin Rheumatol. 2010;29(12):1367–72.

    Article  PubMed  Google Scholar 

  26. Espinosa G, Font J, Tassies D, Vidaller A, Deulofeu R, Lopez-Soto A, et al. Vascular involvement in Behcet’s disease: relation with thrombophilic factors, coagulation activation, and thrombomodulin. Am J Med. 2002;112(1):37–43.

    Article  CAS  PubMed  Google Scholar 

  27. Leiba M, Seligsohn U, Sidi Y, Harats D, Sela BA, Griffin JH, et al. Thrombophilic factors are not the leading cause of thrombosis in Behcet’s disease. Ann Rheum Dis. 2004;63(11):1445–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Chambers JC, Haskard DO, Kooner JS. Vascular endothelial function and oxidative stress mechanisms in patients with Behcet’s syndrome. J Am Coll Cardiol. 2001;37(2):517–20.

    Article  CAS  PubMed  Google Scholar 

  29. Erkan F, Gül A, Tasali E. Pulmonary manifestations of Behçet’s disease. Thorax. 2001;56:572–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  30. Hirohata S, Kikuchi H. Histopathology of the ruptured pulmonary artery aneurysm in a patient with Behçet’s disease. Clin Exp Rheumatol. 2009;27:S91–5.

    CAS  PubMed  Google Scholar 

  31. Ergun T, Gürbüz O, Harvell J, et al. The histopathology of pathergy: a chronologic study of skin hyperreactivity in Behçet’s disease. Int J Dermatol. 1998;37:929–93.

    Article  CAS  PubMed  Google Scholar 

  32. Matsumura N, Mizushima Y. Leucocyte movement and colchicine treatment in Behcet’s disease. Lancet. 1975;2:813.

    Article  CAS  PubMed  Google Scholar 

  33. Eksioglu-Demiralp E, Direskeneli H, Kibaroglu A, Yavuz S, Ergun T, Akoglu T. Neutrophil activation in Behçet’s disease. Clin Exp Rheumatol. 2001;19:S19–24.

    CAS  PubMed  Google Scholar 

  34. Saruhan-Direskeneli G, Yentur SP, Akman-Demir G, Isik N, Serdaroglu P. Cytokines and chemokines in neuro-Behcet’s disease compared to multiple sclerosis and other neurological diseases. J Neuroimmunol. 2003;145:127–34.

    Article  CAS  PubMed  Google Scholar 

  35. Kawakami T, Ohashi S, Kawa Y, Takahama H, Ito M, Soma Y, et al. Elevated serum granulocyte colony stimulating factor levels in patients with active phase of sweet syndrome and patients with active behcet disease: implication in neutrophil apoptosis dysfunction. Arch Dermatol. 2004;140:570–4.

    Article  CAS  PubMed  Google Scholar 

  36. Yavuz S, Akdeniz T, Hancer V, Bicakcigil M, Can M, Yanikkaya-Demirel G. Dual effect of testosterone in Behcet’s disease: implications for a role in disease pathogenesis. Genes Immun. 2016;17(6):335–41.

    Article  CAS  PubMed  Google Scholar 

  37. Ørensen OE, Borregaard N. Neutrophil extracellular traps—the dark side of neutrophils. J Clin Invest. 2016;126:1612–20.

    Article  Google Scholar 

  38. Lee KH, Kronbichler A, Park DD, Park Y, Moon H, Kim H, et al. Neutrophil extracellular traps (NETs) in autoimmune diseases: a comprehensive review. Autoimmun Rev. 2017;16(11):1160–73.

    Article  CAS  PubMed  Google Scholar 

  39. Martinod K, Wagner DD. Thrombosis: tangled up in nets. Blood. 2014;123:2768–76.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  40. Le Joncour A, Martos R, Loyau S, Lelay N, Dossier A, Cazes A, et al. Critical role of neutrophil extracellular traps (NETs) in patients with Behcet’s disease. Ann Rheum Dis. 2019;78(9):1274–82.

    Article  PubMed  Google Scholar 

  41. Becatti M, Emmi G, Silvestri E, Bruschi G, Ciucciarelli L, Squatrito D, et al. Neutrophil activation promotes fibrinogen oxidation and thrombus formation in Behçet disease. Circulation. 2016;133:302–11.

    Article  CAS  PubMed  Google Scholar 

  42. Mittal M, Siddiqui MR, Tran K, Reddy SP, Malik AB. Reactive oxygen species in inflammation and tissue injury. Antioxid Redox Signal. 2014;20:1126–67.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  43. Soehnlein O. Multiple roles for neutrophils in atherosclerosis. Circ Res. 2012;110:875–88.

    Article  CAS  PubMed  Google Scholar 

  44. van Montfoort ML, Stephan F, Lauw MN, Hutten BA, Van Mierlo GJ, Solati S, et al. Circulating nucleosomes and neutrophil activation as risk factors for deep vein thrombosis. Arterioscler Thromb Vasc Biol. 2013;33:147–51.

    Article  PubMed  Google Scholar 

  45. Emmi G, Becatti M, Bettiol A, Hatemi G, Prisco D, Fiorillo C. Behçet’s syndrome as a model of thrombo-inflammation: the role of neutrophils. Front Immunol. 2019;10:1085.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  46. Vallier L, Cointe S, Lacroix R, Bonifay A, Judicone C, Dignat-George F, Kwaan HC. Microparticles and fibrinolysis. Semin Thromb Hemost. 2017;43(2):129–34.

    Article  CAS  PubMed  Google Scholar 

  47. Mejía JC, Ortiz T, Tàssies D, Solanich X, Vidaller A, Cervera R, et al. Procoagulant microparticles are increased in patients with Behçet’s disease but do not define a specific subset of clinical manifestations. Clin Rheumatol. 2016;35(3):695–9.

    Article  PubMed  Google Scholar 

  48. Macey M, Hagi-Pavli E, Stewart J, Wallace GR, Stanford M, Shirlaw P, et al. Age, gender and disease-related platelet and neutrophil activation ex vivo in whole blood samples from patients with Behçet’s disease. Rheumatology (Oxford). 2011;50(10):1849–59.

    Article  CAS  Google Scholar 

  49. Khan E, Ambrose NL, Ahnström J, Kiprianos AP, Stanford MR, Eleftheriou D, et al. A low balance between microparticles expressing tissue factor pathway inhibitor and tissue factor is associated with thrombosis in Behçet’s Syndrome. Sci Rep. 2016;6:38104.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  50. Seyahi E, Cakmak OS, Tutar B, Arslan C, Dikici AS, Sut N, et al. Clinical and Ultrasonographic evaluation of lower-extremity vein thrombosis in Behcet syndrome: an observational study. Medicine (Baltimore). 2015;94(44):e1899.

    Article  Google Scholar 

  51. Alibaz-Oner F, Aldag B, Aldag M, Unal AU, Mutiş A, Toptas T, Ergun T, Direskeneli H. Post-thrombotic syndrome and venous disease-specific quality of life in patients with vascular Behçet’s disease. J Vasc Surg Venous Lymphat Disord. 2016;4(3):301–6.

    Article  PubMed  Google Scholar 

  52. Ozguler Y, Hatemi G, Cetinkaya F, Tascilar K, Hamuryudan V, Ugurlu S, Seyahi E, Yazici H, Melikoglu M. Clinical course of acute deep vein thrombosis of the legs in Behçet’s syndrome. Rheumatology (Oxford). 2020;59(4):799–806.

    Article  Google Scholar 

  53. Kahn SR, Ginsberg JS. Relationship between deep venous thrombosis and the postthrombotic syndrome. Arch Intern Med. 2004;164:17–26.

    Article  PubMed  Google Scholar 

  54. Kahn SR, Hirsch A, Shrier I. Effect of post-thrombotic syndrome on health-related quality of life after deep venous thrombosis. Arch Intern Med. 2002;162:1144–8.

    Article  PubMed  Google Scholar 

  55. Kahn SR, Lamping DL, Ducruet T, Arsenault L, Miron MJ, Roussin A, et al. VEINES-QOL/Sym questionnaire was a reliable and valid disease-specific quality of life measure for deep venous thrombosis. J Clin Epidemiol. 2006;59:1049–56.

    Article  PubMed  Google Scholar 

  56. Jung JY, Kim DY, Bang D. Leg ulcers in Behcet’s disease. Br J Dermatol. 2008;158(1):178–9.

    PubMed  Google Scholar 

  57. Ozguler Y, Kutlubay Z, Dikici AS, Melikoglu M, Mat C, Yazici H, et al. Leg ulcers in Behçet’s syndrome: an observational survey in 24 patients. Arthritis Rheumatol. 2018;70(Suppl 10):9.

    Google Scholar 

  58. Ambrose N, Pierce IT, Gatehouse PD, Haskard DO, Firmin DN. Magnetic resonance imaging of vein wall thickness in patients with Behçet’s syndrome. Clin Exp Rheumatol. 2014;32:S99–102.

    PubMed  Google Scholar 

  59. Boulon C, Skopinski S, Constans J. Vein inflammation and ultrasound in Behçet’s syndrome. Rheumatology (Oxford). 2016;55:1750.

    Article  Google Scholar 

  60. Alibaz-Oner F, Mutis A, Ergelen R, Erturk Z, Ergun E, Direskeneli D. Venous vessel wall thickness in lower extremity is increased in male Behcet’s disease patients without vascular involvement. Rheumatology (Oxford). 2017;56(Suppl 3).

    Google Scholar 

  61. Alibaz-Oner F, Ergelen R, Mutis A, Erturk Z, Asadov R, Mumcu G, et al. Venous vessel wall thickness in lower extremity is increased in male patients with Behçet’s disease. Clin Rheumatol. 2019;38(5):1447–51.

    Article  PubMed  Google Scholar 

  62. Seyahi E, Gjoni M, Durmaz ES, Durmaz EŞ, Akbaş S, Sut N, et al. Increased vein wall thickness in Behçet disease. J Vasc Surg Venous Lymphat Disord. 2019;7(5):677–684.e2.

    Article  PubMed  Google Scholar 

  63. Kansu E, Ozer FL, Akalin E, Guler Y, Zileli T, Tanman E, et al. Behcet’s syndrome with obstruction of the venae cavae. A report of seven cases. Q J Med. 1972;41(162):151–68.

    CAS  PubMed  Google Scholar 

  64. Houman H, Lamloum M, Ben Ghorbel I, Khiari-Ben Salah I, Miled M. Vena cava thrombosis in Behcet’s disease. Analysis of a series of 10 cases. Ann Med Interne (Paris). 1999;150(8):587–90.

    Google Scholar 

  65. Benjilali L, Harmouche H, Alaoui-Bennesser H, Mezalek ZT, Adnaoui M, Aouni M, et al. Chylothorax and chylopericardium in a young man with Behcet’s disease. Joint Bone Spine. 2008;75(6):743–5.

    Article  PubMed  Google Scholar 

  66. Seyahi E, Caglar E, Ugurlu S, Kantarci F, Hamuryudan V, Sonsuz A, Melikoglu M, Yurdakul S, Yazici H. 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.

    Article  PubMed  Google Scholar 

  67. 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.

    Article  PubMed  PubMed Central  Google Scholar 

  68. Sakr MA, Reda MA, Ebada HE, Abdelmoaty AS, Hefny ZM, Ibrahim ZH, et al. Characteristics and outcome of primary Budd-Chiari syndrome due to Behçet’s syndrome. Clin Res Hepatol Gastroenterol. 2019. pii: S2210-7401(19)30249-9.

    Google Scholar 

  69. Akman-Demir G, Serdaroglu P, Tasçi B. Clinical patterns of neurological involvement in Behçet’s disease: evaluation of 200 patients. The Neuro-Behçet Study Group. Brain. 1999;122(Pt 11):2171–82.

    Article  PubMed  Google Scholar 

  70. 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). 2019;58(4):600–8.

    Article  CAS  Google Scholar 

  71. 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.

    Article  CAS  PubMed  Google Scholar 

  72. Yesilot N, Bahar S, Yilmazer S, Mutlu M, Kurtuncu M, Tuncay R, Coban O, Akman-Demir G. Cerebral venous thrombosis in Behçet’s disease compared to those associated with other etiologies. J Neurol. 2009;256(7):1134–42.

    Article  CAS  PubMed  Google Scholar 

  73. 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.

    Article  CAS  PubMed  Google Scholar 

  74. Hamuryudan V, Er T, Seyahi E, Akman C, Tüzün H, Fresko I, et al. Pulmonary artery aneurysms in Behçet syndrome. Am J Med. 2004;117:867–70.

    Article  PubMed  Google Scholar 

  75. Seyahi E, Melikoglu M, Akman C, Hamuryudan V, Ozer H, Hatemi G, et al. Pulmonary artery involvement and associated lung disease in Behcet disease: a series of 47 patients. Medicine (Baltimore). 2012;91(1):35–48.

    Article  Google Scholar 

  76. Uzun O, Akpolat T, Erkan L. Pulmonary vasculitis in behcet disease: a cumulative analysis. Chest. 2005;127(6):2243–53.

    Article  PubMed  Google Scholar 

  77. Yuan S. Pulmonary artery aneurysms in Behçet disease. J Vasc Bras. 2014;13(3):217–28.

    Article  Google Scholar 

  78. Tunaci M, Ozkorkmaz B, Tunaci A, Gul A, Engin G, Acunas B. CT findings of pulmonary artery aneurysms during treatment for Behcet’s disease. AJR Am J Roentgenol. 1999;172(3):729–33.

    Article  CAS  PubMed  Google Scholar 

  79. Yildizeli SO, Yanartas M, Tas S, Direskeneli H, Mutlu B, Ceyhan B, et al. Outcomes of patients with Behcet’s syndrome after pulmonary endarterectomy. Thorac Cardiovasc Surg. 2018;66(2):187–92.

    Article  PubMed  Google Scholar 

  80. 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;55:157–63.

    Article  PubMed  Google Scholar 

  81. Chae EJ, Do KH, Seo JB, Park SH, Kang JW, Jang YM, et al. Radiologic and clinical findings of Behcet disease: comprehensive review of multisystemic involvement. Radiographics. 2008;28(5):e31.

    Article  PubMed  Google Scholar 

  82. Tuzun H, Besirli K, Sayin A, Vural FS, Hamuryudan V, Hizli N, et al. Management of aneurysms in Behcet’s syndrome: an analysis of 24 patients. Surgery. 1997;121(2):150–6.

    Article  CAS  PubMed  Google Scholar 

  83. Kwon TW, Park SJ, Kim HK, Yoon HK, Kim GE, Yu B. Surgical treatment result of abdominal aortic aneurysm in Behçet’s disease. Eur J Vasc Endovasc Surg. 2008;35:173–80.

    Article  PubMed  Google Scholar 

  84. 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.

    Article  Google Scholar 

  85. Emmungil H, Yaşar Bilge NŞ, Küçükşahin O, Kılıç L, Okutucu S, Gücenmez S, et al. A rare but serious manifestation of Behçet’s disease: intracardiac thrombus in 22 patients. Clin Exp Rheumatol. 2014;32(4 Suppl 84):S87–92. Epub 2014 Jul 28.

    PubMed  Google Scholar 

  86. Lee I, Park S, Hwang I, Kim MJ, Nah SS, Yoo B, et al. Cardiac Behcet disease presenting as aortic valvulitis/aortitis or right heart inflammatory mass: a clinicopathologic study of 12 cases. Am J Surg Pathol. 2008;32(3):390–8.

    Article  PubMed  Google Scholar 

  87. Geri G, Wechsler B, Thi Huong DL, Isnard R, Piette JC, Amoura Z, et al. Spectrum of cardiac lesions in Behcet disease: a series of 52 patients and review of the literature. Medicine (Baltimore). 2012;91(1):25–34.

    Google Scholar 

  88. Fei Y, Li X, Lin S, Song X, Wu Q, Zhu Y, et al. Major vascular involvement in Behcet’s disease: a retrospective study of 796 patients. Clin Rheumatol. 2013;32(6):845–52.

    Article  PubMed  Google Scholar 

  89. Farouk H, Zayed HS, El-Chilali K. Cardiac findings in patients with Behcet’s disease: facts and controversies. Anatol J Cardiol. 2016;16(7):529–33.

    PubMed  PubMed Central  Google Scholar 

  90. Tutar B, Kantarci F, Cakmak OS, Yazici H, Seyahi E. Assessment of deep venous thrombosis in the lower extremity in Behçet’s syndrome: MR venography versus Doppler ultrasonography. Intern Emerg Med. 2019;14(5):705–11.

    Article  PubMed  Google Scholar 

  91. Tunaci A, Berkmen YM, Gokmen E. Thoracic involvement in Behcet’s disease: pathologic, clinical, and imaging features. AJR Am J Roentgenol. 1995;164(1):51–6.

    Article  CAS  PubMed  Google Scholar 

  92. Bonneville F. Imaging of cerebral venous thrombosis. Diagn Interv Imaging. 2014;95(12):1145–50.

    Article  CAS  PubMed  Google Scholar 

  93. Bradbury AW, Milne AA, Murie JA. Surgical aspects of Behçet’s disease. Br J Surg. 1994;81:1712–21.

    Article  CAS  PubMed  Google Scholar 

  94. Esatoglu SN, Seyahi E, Ugurlu S, Gulsen F, Akman C, Cantasdemir M, et al. Bronchial artery enlargement may be the cause of recurrent haemoptysis in Behcet’s syndrome patients with pulmonary artery involvement during follow-up. Clin Exp Rheumatol. 2016;34(6 Suppl 102):92–6.

    PubMed  Google Scholar 

  95. Mehdipoor G, Davatchi F, Ghoreishian H, Arjmand Shabestari A. Imaging manifestations of Behcet’s disease: key considerations and major features. Eur J Radiol. 2018;98:214–25.

    Article  PubMed  Google Scholar 

  96. Cho SB, Yun M, Lee JH, Kim J, Shim WH, Bang D. Detection of cardiovascular system involvement in Behcet’s disease using fluorodeoxyglucose positron emission tomography. Semin Arthritis Rheum. 2011;40(5):461–6.

    Article  PubMed  Google Scholar 

  97. Trad S, Bensimhon L, El Hajjam M, Chinet T, Wechsler B, Saadoun D. 18F-fluorodeoxyglucose-positron emission tomography scanning is a useful tool for therapy evaluation of arterial aneurysm in Behcet’s disease. Joint Bone Spine. 2013;80(4):420–3.

    Article  PubMed  Google Scholar 

  98. International Study Group for Behçet’s Disease. Criteria for diagnosis of Behçet’s disease. Lancet. 1990;335:1078–80.

    Google Scholar 

  99. The International Criteria for Behçet’s Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. International Team for the Revision of the International Criteria for Behçet’s Disease (ITR-ICBD). J Eur Acad Dermatol Venereol. 2014;28(3):338–47.

    Google Scholar 

  100. Kirino Y, Ideguchi H, Takeno M, Suda A, Higashitani K, Kunishita Y, et al. Continuous evolution of clinical phenotype in 578 Japanese patients with Behçet’s disease: a retrospective observational study. Arthritis Res Ther. 2016;18:217.

    Article  PubMed  PubMed Central  Google Scholar 

  101. Hatemi G, Christensen R, Bang D, Bodaghi B, Celik AF, Fortune F, et al. 2018 update of the EULAR recommendations for the management of Behcet’s syndrome. Ann Rheum Dis. 2018;77(6):808–18.

    PubMed  Google Scholar 

  102. Hamuryudan V, Ozyazgan Y, Hizli N, et al. Azathioprine in Behcet’s syndrome: effects on long-term prognosis. Arthritis Rheum. 1997;40:769–74.

    Article  CAS  PubMed  Google Scholar 

  103. Emmi G, Vitale A, Silvestri E, Boddi M, Becatti M, Fiorillo C, et al. Adalimumab-based treatment versus disease-modifying antirheumatic drugs for venous thrombosis in Behcet’s syndrome: a retrospective study of seventy patients with vascular involvement. Arthritis Rheumatol. 2018;70(9):1500–7.

    Article  CAS  PubMed  Google Scholar 

  104. Adler S, Baumgartner I, Villiger PM. Behcet’s disease: successful treatment with infliximab in 7 patients with severe vascular manifestations. A retrospective analysis. Arthritis Care Res (Hoboken). 2012;64:607–11.

    Article  CAS  Google Scholar 

  105. Vitale A, Emmi G, Lopalco G, Gentileschi S, Silvestri E, Fabiani C, et al. Adalimumab effectiveness in Behçet’s disease: short and long-term data from a multicenter retrospective observational study. Clin Rheumatol. 2017;36(2):451–5.

    Google Scholar 

  106. Chan E, Sangle SR, Coghlan JG, D’Cruz DD. Pulmonary artery aneurysms in Behçet’s disease treated with anti-TNFα: a case series and review of the literature. Autoimmun Rev. 2016;15(4):375–8.

    Google Scholar 

  107. Desbois AC, Biard L, Addimanda O, Lambert M, Hachulla E, Launay D, et al. Efficacy of anti-TNF alpha in severe and refractory major vessel involvement of Behcet’s disease: a multicenter observational study of 18 patients. Clin Immunol. 2018;197:54–9.

    Article  CAS  PubMed  Google Scholar 

  108. Aksoy A, Yazici A, Omma A, Cefle A, Onen F, Tasdemir U, et al. Efficacy of TNFα inhibitors for refractory vascular Behçet’s disease: a multicenter observational study of 27 patients and a review of the literature. Int J Rheum Dis. 2020;23(2):256–61.

    Article  PubMed  Google Scholar 

  109. Cantarini L, Vitale A, Scalini P, Dinarello CA, Rigante D, Franceschini R, et al. Anakinra treatment in drug-resistant Behcet’s disease: a case series. Clin Rheumatol. 2015;34:1293–301.

    Article  PubMed  Google Scholar 

  110. Mohammad AJ, Smith RM, Chow YW, Chaudhry AN, Jayne DR. Alemtuzumab as remission induction therapy in Behçet disease: a 20-year experience. J Rheumatol. 2015;42(10):1906–13.

    Article  CAS  PubMed  Google Scholar 

  111. Ding Y, Li C, Liu J, Yu X, Wang Y, Shi J, et al. Tocilizumab in the treatment of severe and/or refractory vasculo-Behçet’s disease: a single-centre experience in China. Rheumatology (Oxford). 2018;57(11):2057–9.

    Google Scholar 

  112. 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.

    Google Scholar 

  113. Hatemi G, Christensen R, Bang D, Bodaghi B, Celik AF, Fortune F, et al. 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis. 2018;77(6):808–18.

    PubMed  Google Scholar 

  114. Dincses E, Esatoglu SN, Fresko I, Melikoglu M, Seyahi E. Outcome of invasive procedures for venous thrombosis in Behçet’s syndrome: case series and systematic literature review. Clin Exp Rheumatol. 2019;37 Suppl 121(6):125–31.

    Google Scholar 

  115. Ozguler Y, Hatemi G. Management of Behçet’s syndrome. Curr Opin Rheumatol. 2016;28(1):45–50.

    Article  CAS  PubMed  Google Scholar 

  116. Alpay-Kanitez N, Çelik S, Baltacioğlu F, Içaçan OC, Bes C, Yildizeli B. Endovascular embolisation with Amplatzer vascular plug of ruptured pulmonary artery aneurism in Behçet’s disease. Clin Exp Rheumatol. 2019;37 Suppl 121(6):152–3.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Rheumatology, Department of Internal Medicine, Marmara University, School of Medicine, Istanbul, Turkey

    Fatma Alibaz-Oner & Haner Direskeneli

Authors
  1. Fatma Alibaz-Oner
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Haner Direskeneli
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Rheumatology Unit, Azienda Unita’ Sanitaria Locale IRCCS di Reggio Emilia, Reggio Emilia, Italy

    Carlo Salvarani

  2. Rheumatology Unit, Azienda Unita’ Sanitaria Locale IRCCS di Reggio Emilia, Reggio Emilia, Italy

    Luigi Boiardi

  3. Rheumatology Unit, Azienda Unita’ Sanitaria Locale IRCCS di Reggio Emilia, Reggio Emilia, Italy

    Francesco Muratore

Rights and permissions

Reprints and permissions

Copyright information

© 2021 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Alibaz-Oner, F., Direskeneli, H. (2021). Arterial and Venous Involvement in Behçet’s Disease. In: Salvarani, C., Boiardi, L., Muratore, F. (eds) Large and Medium Size Vessel and Single Organ Vasculitis. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-030-67175-4_20

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-67175-4_20

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-67174-7

  • Online ISBN: 978-3-030-67175-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation