Clinical Rheumatology

, Volume 35, Issue 10, pp 2501–2507 | Cite as

Intracardiac thrombus in patients with Behcet’s disease: clinical correlates, imaging features, and outcome: a retrospective, single-center experience

  • Hui Wang
  • Xiaoxiao Guo
  • Zhuang TianEmail author
  • Yongtai Liu
  • Qian Wang
  • Mengtao Li
  • Xiaofeng Zeng
  • Quan Fang
Original Article


Intracardiac thrombus (ICT) is a rare but serious complication of Behcet’s disease (BD). The study was to report the clinical characteristics, imaging features, treatment, and outcomes of BD patients with ICT in a single tertiary center. A series of 626 BD patients admitted to Peking Union Medical College hospital between April 2003 and April 2013 were reviewed. A total of 12 (1.9 %) BD patients with ICT were included. ICT was presenting manifestation of BD in 5 of the 12 patients (41.7 %) and occurred mostly in men (9/12, 75 %) younger than 40 years. Patients with ICT had more arterial (66.7 vs. 7.3 %, p < 0.001) and venous involvement (75.0 vs. 8.1 %, p < 0.001) compared to those without ICT. Right heart was the most frequent site of ICT (11/12, 91.7 %). Thrombus were hyperechogenic and immobile with a broad-based attachment on echocardiography in most cases (11/12, 91.7 %). Cardiac magnetic resonance (CMR) was performed in four patients. All thrombi showed isointense on T1-weighted image, while all but one were hypointense on T2-weighted image without delayed enhancement. Remission of ICT was associated with therapy of anticoagulant, corticosteroid, and immunosuppressant in most cases (10/12, 83.3 %). There was no death, and relapses of ICT occurred in only one patient after tapering of immunosuppressant during follow-up. A diagnosis of BD should be considered when a young male patient presents with a mass in the right-sided heart accompanied by vascular lesions. Medical therapy especially adequate immunosuppressants might be the first choice for BD patients with ICT.


Behcet’s disease Cardiac magnetic resonance Echocardiography Intracardiac thrombus 


Compliance with ethical standards




  1. 1.
    Sakane T, Takeno M, Suzuki N et al (1999) Behcet’s disease. N Engl J Med 341:1284–1291CrossRefPubMedGoogle Scholar
  2. 2.
    Koc Y, Gullu I, Akpek G et al (1992) Vascular involvement in Behcet’s disease. J Rheumatol 19:402–410PubMedGoogle Scholar
  3. 3.
    Geri G, Wechsler B, Du Thi Huong L et al (2012) Spectrum of cardiac lesions in Behcet disease: a series of 52 patients and review of the literature. Medicine (Baltimore) 91:25–34CrossRefGoogle Scholar
  4. 4.
    Davies JD (1973) Behcet’s syndrome with haemoptysis and pulmonary lesions. J Pathol 109:351–356CrossRefPubMedGoogle Scholar
  5. 5.
    Kajiya T, Anan R, Kameko M et al (2007) Intracardiac thrombus, superior vena cava syndrome, and pulmonary embolism in a patient with Behcet’s disease: a case report and literature review. Heart Vessels 22:278–283CrossRefPubMedGoogle Scholar
  6. 6.
    Zhu YL, Wu QJ, Guo LL et al (2012) The clinical characteristics and outcome of intracardiac thrombus and aortic valvular involvement in Behcet’s disease: an analysis of 20 cases. Clin Exp Rheumatol 30:S40–S45PubMedGoogle Scholar
  7. 7.
    Khammar Z, Berrady R, Boukhrissa A et al (2011) Intracardiac thrombosis in Behcet disease: clinical presentation and outcome of three cases. J Mal Vasc 36:270–273CrossRefPubMedGoogle Scholar
  8. 8.
    Hammami R, Abid L, Frikha F et al (2012) Intracardiac thrombus in a young man: don’t forget Behcet’s disease! Intern Med 51:1865–1867CrossRefPubMedGoogle Scholar
  9. 9.
    Emmungil H, Yasar Bilge NS, Kucuksahin O et al (2014) A rare but serious manifestation of Behcet’s disease: intracardiac thrombus in 22 patients. Clin Exp Rheumatol 32:S87–S92PubMedGoogle Scholar
  10. 10.
    International Study Group for Behcet’s Disease (1990) Criteria for diagnosis of Behcet’s disease. International Study Group for Behcet’s Disease. Lancet 335:1078–1080Google Scholar
  11. 11.
    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:S14–S15Google Scholar
  12. 12.
    International Team for the Revision of the International Criteria for Behcet’s D (2014) The International Criteria for Behcet’s Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol 28:338–347CrossRefGoogle Scholar
  13. 13.
    Yurdakul S, Tuzuner N, Yurdakul I et al (1996) Gastrointestinal involvement in Behcet’s syndrome: a controlled study. Ann Rheum Dis 55:208–210CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Mogulkoc N, Burgess MI, Bishop PW (2000) Intracardiac thrombus in Behcet’s disease: a systematic review. Chest 118:479–487CrossRefPubMedGoogle Scholar
  15. 15.
    Davatchi F, Chams-Davatchi C, Shams H et al. (2015) Adult Behcet’s disease in Iran: analysis of 6075 patients. Int J Rheum Dis. doi: 10.1111/1756-185X.12691
  16. 16.
    Wang LY, Zhao DB, Gu J et al (2010) Clinical characteristics of Behcet’s disease in China. Rheumatol Int 30:1191–1196CrossRefPubMedGoogle Scholar
  17. 17.
    Aksu T, Tufekcioglu O (2015) Intracardiac thrombus in Behcet’s disease: four new cases and a comprehensive literature review. Rheumatol Int 35:1269–1279CrossRefPubMedGoogle Scholar
  18. 18.
    Atzeni F, Sarzi-Puttini P, Doria A et al (2005) Behçet’s disease and cardiovascular involvement. Lupus 14:723–726CrossRefPubMedGoogle Scholar
  19. 19.
    Calamia KT, Schirmer M, Melikoglu M (2011) Major vessel involvement in Behcet’s disease: an update. Curr Opin Rheumatol 23:24–31CrossRefPubMedGoogle Scholar
  20. 20.
    Fernandez-Bello I, Lopez-Longo FJ, Arias-Salgado EG et al (2013) Behcet’s disease: new insight into the relationship between procoagulant state, endothelial activation/damage and disease activity. Orphanet J Rare Dis 8:81CrossRefPubMedPubMedCentralGoogle Scholar
  21. 21.
    Direskeneli H, Keser G, D’cruz D et al (1995) Anti-endothelial cell antibodies, endothelial proliferation and von Willebrand factor antigen in Behcet’s disease. Clin Rheumatol 14:55–61CrossRefPubMedGoogle Scholar
  22. 22.
    Kiraz S, Ertenli I, Ozturk MA et al (2002) Pathological haemostasis and “prothrombotic state” in Behcet’s disease. Thromb Res 105:125–133CrossRefPubMedGoogle Scholar
  23. 23.
    Louali FE, Tamdy A, Soufiani A et al (2010) Cardiac thrombosis as a manifestation of Behcet syndrome. Tex Heart Inst J 37:568–571PubMedPubMedCentralGoogle Scholar
  24. 24.
    Mollet NR, Dymarkowski S, Volders W et al (2002) Visualization of ventricular thrombi with contrast-enhanced magnetic resonance imaging in patients with ischemic heart disease. Circulation 106:2873–2876CrossRefPubMedGoogle Scholar
  25. 25.
    Srichai MB, Junor C, Rodriguez LL et al (2006) Clinical, imaging, and pathological characteristics of left ventricular thrombus: a comparison of contrast-enhanced magnetic resonance imaging, transthoracic echocardiography, and transesophageal echocardiography with surgical or pathological validation. Am Heart J 152:75–84CrossRefPubMedGoogle Scholar
  26. 26.
    Ahn JK, Lee YS, Jeon CH et al (2008) Treatment of venous thrombosis associated with Behcet’s disease: immunosuppressive therapy alone versus immunosuppressive therapy plus anticoagulation. Clin Rheumatol 27:201–205CrossRefPubMedGoogle Scholar
  27. 27.
    Desbois AC, Wechsler B, Resche-Rigon M et al (2012) Immunosuppressants reduce venous thrombosis relapse in Behcet’s disease. Arthritis Rheum 64:2753–2760CrossRefPubMedGoogle Scholar
  28. 28.
    Seyahi E, Yurdakul S (2011) Behcet’s syndrome and thrombosis. Mediterr J Hematol Infect Dis 3, e2011026CrossRefPubMedPubMedCentralGoogle Scholar
  29. 29.
    Owlia MB, Mehrpoor G (2012) Behcet’s disease: new concepts in cardiovascular involvements and future direction for treatment. ISRN Pharmacol 2012:760484PubMedPubMedCentralGoogle Scholar

Copyright information

© International League of Associations for Rheumatology (ILAR) 2016

Authors and Affiliations

  • Hui Wang
    • 1
  • Xiaoxiao Guo
    • 1
  • Zhuang Tian
    • 1
    Email author
  • Yongtai Liu
    • 1
  • Qian Wang
    • 2
  • Mengtao Li
    • 2
  • Xiaofeng Zeng
    • 2
  • Quan Fang
    • 1
  1. 1.Department of Cardiology, Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina
  2. 2.Department of Rheumatology, Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina

Personalised recommendations