Clinical Rheumatology

, Volume 32, Issue 6, pp 845–852 | Cite as

Major vascular involvement in Behçet’s disease: a retrospective study of 796 patients

  • Yunyun Fei
  • Xuemei Li
  • Sen Lin
  • Xiaojun Song
  • Qingjun Wu
  • Yanlin Zhu
  • Xin Gao
  • Wen ZhangEmail author
  • Yan Zhao
  • Xiaofeng Zeng
  • Fengchun Zhang
Original Article


Behçet’s disease (BD) is a multi-systemic inflammatory disorder which can affect all types and sizes of blood vessels. This study aims to evaluate the prevalence and characteristics of vascular involvement in BD. Among 796 patients diagnosed with BD, 102 patients (81 male, 21 female) with vascular involvement were included, whose detailed clinical characteristics were recorded. The diagnosis of vascular lesions was made on clinical signs, by Doppler ultrasonography, and/or angiography using computed tomographic or magnetic resonance techniques. Vascular involvement occurred in 12.8 % of BD patients. Male to female ratio was 3.86:1. Mean age at onset of vascular involvement was 29.5 ± 11.3 years. Vascular lesion was the initial sign of BD in 28 patients, accounting for 27.5 %. Of 102 BD patients with vascular involvement, 72 had venous lesions (70.6 %) and 56 had arterial lesions (54.9 %), among which 26 (25.5 %) patients had both venous and arterial involvements. Female BD patients were more often involved with arterial lesions, whereas male BD patients developed venous lesions more often than females, P = 0.000. The most common type of vascular involvement was deep venous thrombosis in lower extremities (n = 49), other affected venous sites including inferior vena cava, superior vena cava, and cerebral venous. The prominent type of arterial lesions was dilatation (n = 25, including 24 cases of aneurysms); other types included eight cases of occlusion and 23 cases of stenosis. The main locations of arterial lesions were the aorta (n = 19), lower extremity arteries (n = 15), pulmonary arteries (n = 13), coronary arteries (n = 5), and subclavian arteries (n = 5). Compared with those without vascular lesions, ocular involvement, genital ulcers, and arthritis were significantly less frequent among patients with vasculo-BD (23.5 vs 35.2 %, P = 0.024; 54.9 vs 76.5 %, P = 0.000; 19.6 vs 30.5 %, P = 0.026), whereas a higher frequency of cardiac involvement was found in vasculo-BD patients (20.6 vs 3.6 %, P = 0.000). Vascular involvement is a complication in BD patients. This study illustrated that venous lesions are more frequently involved than arterial lesions. Vascular lesions correlated with a high frequency of cardiac involvement and a low incidence of ocular lesions, genital ulcers, and arthritis.


Behçet’s disease China Vascular involvement 



This work was supported by The National Natural Science Foundation of China (no. 81172858; 81202360).




  1. 1.
    Sakane T, Takeno M, Suzuki N et al (1999) Behçet’s disease. N Engl J Med 341(17):1284–1291PubMedCrossRefGoogle Scholar
  2. 2.
    Tursen U, Gurler A, Boyvat A (2003) Evaluation of clinical findings according to sex in 2313 Turkish patients with Behçet’s disease. Int J Dermatol 42(5):346–351PubMedCrossRefGoogle Scholar
  3. 3.
    Bang D, Lee JH, Lee ES et al (2001) Epidemiologic and clinical survey of Behcet’s disease in Korea: the first multicenter study. J Korean Med Sci 16(5):615–618PubMedGoogle Scholar
  4. 4.
    Davatchi F, Shahram F, Chams-Davatchi C et al (2010) Behcet’s disease in Iran: analysis of 6500 cases. Int J Rheum Dis 13(4):367–373PubMedCrossRefGoogle Scholar
  5. 5.
    Zhang Z, Peng J, Hou X et al (2006) Clinical manifestations of Behcet’s disease in Chinese patients. APLAR J Rheumatol 9:244–247CrossRefGoogle Scholar
  6. 6.
    Ideguchi H, Suda A, Takeno M et al (2011) Characteristics of vascular involvement in Behçet’s disease in Japan: a retrospective cohort study. Clin Exp Rheumatol 29(4 Suppl 67):S47–S53PubMedGoogle Scholar
  7. 7.
    Ko GY, Byun JY, Choi BG et al (2000) The vascular manifestations of Behcet’s disease: angiography and CT findings. Br J Radiol 73(876):1270–1274PubMedGoogle Scholar
  8. 8.
    Düzgün N, Ateş A, Aydintuğ OT et al (2006) Characteristics of vascular involvement in Behçet’s disease. Scand J Rheumatol 35(1):65–68PubMedCrossRefGoogle Scholar
  9. 9.
    Tohme A, Aoun N, El-Rassi B et al (2003) Vascular manifestations of Behcet’s disease. Eighteen cases among 140 patients. Jt Bone Spine 70:384–389CrossRefGoogle Scholar
  10. 10.
    Sarica-Kucukoglu R, Akdag-Kose A, KayabalI M et al (2006) Vascular involvement in Behçet’s disease: a retrospective analysis of 2319 cases. Int J Dermatol 45(8):919–921PubMedCrossRefGoogle Scholar
  11. 11.
    Al-Dalaan AN, al Balaa SR, el Ramahi K et al (1994) Behcet’s disease in Saudi Arabia. J Rheumatol 21:658–661PubMedGoogle Scholar
  12. 12.
    Altenburg A, Papoutsis N, Orawa H et al (2006) Epidemiology and clinical manifestations of Adamantiades–Behçet disease in Germany—current pathogenetic concepts and therapeutic possibilities. J Dtsch Dermatol Ges 4(1):49–64PubMedCrossRefGoogle Scholar
  13. 13.
    Seaman G, Pearce R (1997) Disease manifestation in a population drawn from the UK Behcet’s Syndrome Society. In: Hamza M (ed) Behcet's Disease. PUB ADHOUA, Tunisia, pp 196–199Google Scholar
  14. 14.
    Sharqui K, Al-Araji A, Al-Rawi Z (2000) Behcet’s disease in Iraqi patients. A prospective study from a newly established multidiscipline Behcet’s disease clinic. In: Bang D, Lee E, Lee S (eds) Behcet’s disease. Design Mecca, Seoul, pp 60–63Google Scholar
  15. 15.
    Assaad-Khalil S, Kamel F, Ismail E (1997) Starting regional registry for patients with Behcet’s disease in North-West Nile Delta region in Egypt. In: Hamza M (ed) Behcet's Disease. PUB ADHOUA, Tunisia, pp 173–176Google Scholar
  16. 16.
    Madanat W, Fayyad F, Zureikat H et al (2000) Analysis of 150 patients with Behcet’s disease from Jordan. In: Bang D, Lee E, Lee S (eds) Behcet’s disease. Design Mecca, Seoul, pp 941–944Google Scholar
  17. 17.
    Calamia KT, Mazlumzadeh M, Balbanova M et al (2000) Clinical characteristics of United States patients with Behcet’s disease. In: Bang D, Lee E, Lee S (eds) Behcet’s disease. Design Mecca, Seoul, pp 48–51Google Scholar
  18. 18.
    Benamour S, Naji T, Echchilali K et al (2006) Study of 1034 cases of Behcet’s disease. Clin Exp Rheumatol 24(suppl 42):S13Google Scholar
  19. 19.
    B'chir Hamzaoui S, Harmel A, Bouslam K et al (2006) Behcet’s disease in Tunisia. Clinical study of 519 cases. Rev Med Intern 27:742–750CrossRefGoogle Scholar
  20. 20.
    Valesini G, Pivetti Pezzi P, Catarinelli G et al (1991) Clinical manifestations of Behcet's disease in Italy: study of 155 patients at Rome University. In: O'Duffy JD, Kokmen E (eds) Behcet's Disease Basic and Clinical Aspects. Marcel Decker Inc, New York, pp 279–289Google Scholar
  21. 21.
    de Souza-Ramalho P, DAlmeida MF, Freitas JP et al (1991) Incidence and clinical aspects of Behcet’s disease in Portugal. In: O'Duffy JD, Kokmen E (eds) Behcet’s disease basic and clinical aspects. Marcel Decker, New York, pp 291–298Google Scholar
  22. 22.
    Ames PR, Steuer A, Pap A, Denman AM (2001) Thrombosis in Behcet’s disease: a retrospective survey from a single UK centre. Rheumatol (Oxford) 40:652–655CrossRefGoogle Scholar
  23. 23.
    Seyahi E, Yurdakul S (2011) Behçet’s syndrome and thrombosis. Mediterr J Hematol Infect Dis 3(1):e2011026PubMedCrossRefGoogle Scholar
  24. 24.
    Saadoun D, Asli B, Wechsler B et al (2012) Long-term outcome of arterial lesions in Behçet disease: a series of 101 patients. Med (Baltimore) 91(1):18–24CrossRefGoogle Scholar
  25. 25.
    Seyahi E, Melikoglu M, Akman C et al (2012) Pulmonary artery involvement and associated lung disease in Behçet disease: a series of 47 patients. Med (Baltimore) 91(1):35–48CrossRefGoogle Scholar
  26. 26.
    Mizushima Y, Inaba G, Mimura Y et al (1988) Diagnostic criteria for Behcet’s disease in 1987, and guideline for treating Behcet’s disease. Saishin Igaku 43:391–393Google Scholar
  27. 27.
    International Study Group for Behcet’s Disease (1990) Criteria for diagnosis of Behcet’s disease. Lancet 335:1078–1080Google Scholar
  28. 28.
    International Team for the Revision of the International Criteria for Behcet’s Disease (2006) Revision of the International Criteria for Behcet’s Disease (ICBD). Clin Exp Rheumatol 24(Suppl 42):S14–S15Google Scholar
  29. 29.
    Chen SP, Zhang X-Q (1980) Some special clinical manifestations of Behcet’s disease—report of illustrative cases and review of literature (author’s transl). Chin J Intern Med 19(1):15–22Google Scholar
  30. 30.
    Zhang Z, He F, Shi Y (2012) Behcet’s disease seen in China: analysis of 334 cases. Rheumatol Int. doi: 10.1007/s00296-012-2384-6
  31. 31.
    Erkan F, Gül A, Tasali E (2001) Pulmonary manifestations of Behçet’s disease. Thorax 56(7):572–578PubMedCrossRefGoogle Scholar
  32. 32.
    Hiller N, Lieberman S, Chajek-Shaul T et al (2004) Thoracic manifestations of Behçet disease at CT. RadioGraphics 24(3):801–808PubMedCrossRefGoogle Scholar
  33. 33.
    Raz I, Okon E, Chajek-Shaul T (1989) Pulmonary manifestations in Behçet’s syndrome. Chest 95(3):585–589PubMedCrossRefGoogle Scholar
  34. 34.
    Hamuryudan V, Yurdakul S, Moral F et al (1994) Pulmonary arterial aneurysms in Behçet’s syndrome: a report of 24 cases. Br J Rheumatol 33(1):48–51PubMedCrossRefGoogle Scholar
  35. 35.
    Geri G, Wechsler B, Thi Huongdu L et al (2012) Spectrum of cardiac lesions in Behçet disease: a series of 52 patients and review of the literature. Med (Baltimore) 91(1):25–34CrossRefGoogle Scholar
  36. 36.
    Emad Y, Abdel-Razek N, Gheita T et al (2007) Multislice CT pulmonary findings in Behçet's disease (report of 16 cases). Clin Rheumatol 26(6):879–884PubMedCrossRefGoogle Scholar
  37. 37.
    Schoepf UJ, Kessler MA, Rieger CT et al (2001) Multislice CT imaging of pulmonary embolism. Eur Radiol 11(11):2278–2286PubMedCrossRefGoogle Scholar
  38. 38.
    Hamuryudan V, Hatemi G, Tascilar K et al (2010) Prognosis of Behcet’s syndrome among men with mucocutaneous involvement at disease onset: long-term outcome of patients enrolled in a controlled trial. Rheumatol (Oxford) 49(1):173–177CrossRefGoogle Scholar
  39. 39.
    Alpagut U, Ugurlucan M, Dayioglu E (2007) Major arterial involvement and review of Behcet’s disease. Ann Vasc Surg 21(2):232–239PubMedCrossRefGoogle Scholar

Copyright information

© Clinical Rheumatology 2013

Authors and Affiliations

  • Yunyun Fei
    • 1
  • Xuemei Li
    • 2
  • Sen Lin
    • 3
  • Xiaojun Song
    • 4
  • Qingjun Wu
    • 1
  • Yanlin Zhu
    • 5
  • Xin Gao
    • 6
  • Wen Zhang
    • 1
    Email author
  • Yan Zhao
    • 1
  • Xiaofeng Zeng
    • 1
  • Fengchun Zhang
    • 1
  1. 1.Department of RheumatologyPeking Union Medical HospitalBeijingChina
  2. 2.Respiratory DepartmentBeijing General Hospital of Jingmei GroupBeijingChina
  3. 3.Department of RheumatologyZaozhuang Hospital of Traditional Chinese MedicineZaozhuangChina
  4. 4.Department of Vascular SurgeryPeking Union Medical HospitalBeijingChina
  5. 5.Department of CardiologyPeking Union Medical HospitalBeijingChina
  6. 6.Department of RadiologyPeking Union Medical HospitalBeijingChina

Personalised recommendations