Abstract
Behçet disease (BD) is a chronic multisystem disorder with vasculitis underlying its systemic manifestations. Cardiac involvement and particularly left ventricular intracardiac thrombus are rarely diagnosed in the course of BD and are often associated with poor prognosis. The causes of intracardiac thrombi are unknown. It is plausible that specific proinflammatory pathways resulting in the endothelial cell injury and hypercoagulation contribute to the formation of thrombotic masses in the heart. Known thrombophilic factors such as methylenetetrahydrofolate reductase gene mutations, factor V Leiden mutation, proteins S and C, antithrombin III, activated protein C resistance, and antiphospholipid antibodies may contribute to the formation of intracardiac thrombi in BD. We report a case of a 24-year-old male patient with BD presented with left ventricular thrombus. Transthoracic echocardiography allowed to describe and monitor such a rare cardiac manifestation of the disease. A combination of high-dose corticosteroid and azathioprine successfully dissolved intracardiac thrombus within ten days without anticoagulation.
Similar content being viewed by others
References
Kitaichi N, Miyazaki A, Iwata D, Ohno S, Stanford MR, Chams H (2007) Ocular features of Behcet’s disease: an international collaborative study. Br J Ophthalmol 91(12):1579–1582
Alekberova ZS (2013) Behçet’s disease. Rheumatol Sci Pract 51(1): 52–58. http://www.rsp-j.ru/index.php/rsp/article/view/1112/785
La Regina M, Gasparyan AY, Orlandini F, Prisco D (2010) Behçet’s disease as a model of venous thrombosis. Open Cardiovasc Med J 4:71–77
Lakpanhal S, Tani K, Lie JT, Katoh K, Ishigatsubo Y, Ohokubo T (1985) Pathologic features of Behçet’s syndrome: a review of Japanese autopsy registry data. Hum Pathol 16:790–795
Wechsler B, Du LT, Kiffer E (1999) Cardiovascular manifestations of Behçet’s disease. Ann Med Interne 150:542–554
Zhang Z, He F, Shi Y (2013) Behcet’s disease seen in China: analysis of 334 cases. Rheumatol Int 33(3):645–648
Saadoun D, Wechsler B, Desseaux K, Le Thi Huong D, Amoura Z, Resche-Rigon M, Cacoub P (2010) Mortality in Behçet’s disease. Arthritis Rheum 62:2806–2812
Sarica-Kucukoglu R, Akdag-Kose A, Kayabal IM, Yazganoglu KD, Disci R, Erzengin D, Azizlerli G (2006) Vascular involvement in Behçet’s disease: a retrospective analysis of 2319 cases. Int J Dermatol 45:919–921
Koc Y, Gullu I, Akpek G, Akpolat T, Kansu E, Kiraz S, Batman F, Kansu T, Balkanci F, Akkaya S (1992) Vascular involvement in Behçet’s disease. J Rheumatol 19:402–410
Houman MH, Neffati H, Braham A, Harzallah O, Khanfir M, Miled M, Hamzaoui K (2007) Behçet’s disease in Tunisia: demographic, clinical and genetic aspects in 260 patients. Clin Exp Rheumatol 25:S58–S64
Tohme A, Aoun N, El-Rassi B, Ghayad E (2003) Vascular manifestations of Behçet’s disease: eighteen cases among 140 patients. Joint Bone Spine 70:384–389
Kuzu MA, Ozaslan C, Koksoy C, Gurler A, Tuzuner A (1994) Vascular involvement in Behçet’s disease: 8-year audit. World J Surg 18:948–953
Duzgun N, Ates A, Aydintug OT, Demir O, Olmez U (2006) Characteristics of vascular involvement in Behçet’s disease. Scand J Rheumatol 35:65–68
Emmungil H, Yaşar Bilge NŞ, Küçükşahin O, Kiliç L, Okutucu S, Gücenmez S, Kalyoncu U, Kaşifoǧlu T, Turgay M, Aksu K (2014) A rare but serious manifestation of Behçet’s disease: intracardiac thrombus in 22 patients. Clin Exp Rheumatol 32(Suppl 84):S87–S92
Vivante A, Bujanover Y, Jacobson J, Padeh S, Berkun Y (2009) Intracardiac thrombus and pulmonary aneurysms in an adolescent with Behçet disease. Rheumatol Int 29(5):575–577
Alkaabi JK, Pathare A (2011) Pattern and outcome of vascular involvement of Omani patients with Behcet’s disease. Rheumatol Int 31(6):731–735
Hammami R, Abid L, Frikha F, Marzouk S, Tounsi A, Frikha Z, Hentati M, Bahloul Z, Kammoun S (2012) Intracardiac thrombus in a young man: don’t forget Behçet’s disease! Intern Med 51(14):1865–1867
Tayer-Shifman OE, Seyahi E, Nowatzky J, Ben-Chetrit E (2012) Major vessel thrombosis in Behçet’s disease: the dilemma of anticoagulant therapy—the approach of rheumatologists from different countries. Clin Exp Rheumatol 30(5):735–740
Dogan SM, Birdane A, Korkmaz C, Ata N, Timuralp B (2007) Right ventricular thrombus with Behçet’s syndrome: successful treatment with warfarin and immunosuppressive agents. Tex Heart Inst J 34(3):360–362
Louali FE, Tamdy A, Soufiani A, Oukerraj L, Omari D, Bounjoum F, Mekouar F, Tazi-Mezalek Z, Bouhouch R, Zarzur J, Cherti M (2010) Cardiac thrombosis as a manifestation of Behçet syndrome. Tex Heart Inst J 37(5):568–571
Canpolat U, Yorgun H, Akdoğan A, Aytemir K (2012) Successful treatment of intracardiac and pulmonary thrombi in Behçet’s disease with oral anticoagulant and immunosuppressive therapy. Acta Medica (Hradec Kralove) 55(4):186–188
Cocco G, Gasparyan AY (2010) Behçet’s disease: an insight from a cardiologist’s point of view. Open Cardiovasc Med J 4:63–70
Ishida C, Furui E, Sakashita Y, Yamada M (2005) Embolic stroke with a patent foramen ovale and Behcet’s disease. Intern Med 44(4):326–327
Madanat WY, Prokaeva TB, Kotel’nikova GP, Alekberova ZS (1993) Endocarditis with left atrial thrombus formation in Behçet’s disease mimicking myxoma. J Rheumatol 20:1982–1984
International Study Group for Behçet’s Disease (1990) Criteria for diagnosis of Behçet’s disease. Lancet 335:1078–1080
Farouk H, Chilali KE, Said K, Sakr B, Salah H, Mahmoud G, Sorour K (2014) Value of certain echocardiographic findings in the initial suspicion of Behçet’s disease. Echocardiography 31(8):924–930
Mogulkoc N, Burgess MI, Bishop PW (2000) Intracardiac thrombus in Behçet’s disease: a systematic review. Chest 118(2):479–487
Chiari E, Fracassi F, D’Aloia A, Vizzardi E, Zanini G, Rocca P, Metra M, Cas LD (2008) Right ventricular thrombus and pulmonary thromboembolism/thrombosis in Behçet’s disease: a case report. J Am Soc Echocardiogr 21:1079
Núñez-Cabarcas E, López-Ruiz N, Ramírez-Rincón A (2014) Diagnostic dilemma: intracardiac mass in a woman with Behçet’s syndrome. Arch Cardiol Mex 84(4):273–275
Farouk H (2014) Behçet’s disease, echocardiographers, and cardiac surgeons: together is better. Echocardiography 31(6):783–787
Leibowitz D, Korem M, Livovsky DM, Peleg H, Chajek-Shaul T, Hiller N (2014) Intracardiac mass as initial cardiac manifestation of Behçet’s disease: think before you cut. Echocardiography 31(8):E243–E246
Kajiya T, Anan R, Kameko M, Mizukami N, Minagoe Sh, Hamasaki Sh, Maruyama I, Sakata R, Tei Ch (2007) Intracardiac thrombus, superior vena cava syndrome, and pulmonary embolism in a patient with Behçet’s disease: a case report and literature review. Heart Vessels 22:278–283
Aydìntug AO, Tokgöz G, D’Cruz DP, Gürler A, Cervera R, Düzgün N, Atmaca LS, Khamashta MA, Hughes GR (1993) Antibodies to endothelial cells in patients with Behçet’s disease. Clin Immunol Immunopathol 67(2):157–162
Florey OJ, Johns M, Esho OO, Mason JC, Haskard DO (2007) Antiendothelial cell antibodies mediate enhanced leukocyte adhesion to cytokine-activated endothelial cells through a novel mechanism requiring cooperation between Fc{gamma}RIIa and CXCR1/2. Blood 109(9):3881–3889
Tunc SE, Aksu K, Keser G, Oksel F, Doganavsargil E, Pirildar T, Turk T, Terzioglu E, Huseyinov A (2005) Platelet-activating factor and P-selectin activities in thrombotic and nonthrombotic Behçet’s patients. Rheumatol Int 25(5):326–331
Macey M, Hagi-Pavli E, Stewart J, Wallace GR, Stanford M, Shirlaw P, Fortune F (2011) Age, gender and disease-related platelet and neutrophil activation ex vivo in whole blood samples from patients with Behçet’s disease. Rheumatology (Oxford) 50(10):1849–1859
Fernández-Bello I, López-Longo FJ, Arias-Salgado EG, Jiménez-Yuste V, Butta NV (2013) Behçet’s disease: new insight into the relationship between procoagulant state, endothelial activation/damage and disease activity. Orphanet J Rare Dis 8:81
Kiraz S, Ertenli I, Oztürk MA, Haznedaroğlu IC, Celik I, Calgüneri M (2002) Pathological haemostasis and “prothrombotic state” in Behçet’s disease. Thromb Res 105(2):125–133
Caramaschi P, Poli G, Bonora A, Volpe A, Tinazzi I, Pieropan S, Bambara LM, Biasi D (2010) A study on thrombophilic factors in Italian Behcet’s patients. Joint Bone Spine 77(4):330–334
Hiwarkar P, Stasi R, Sutherland G, Shannon M (2010) Deep vein and intracardiac thrombosis during the post-partum period in Behçet’s disease. Int J Hematol 91(4):679–686
Chang JE, Lee YH, Lee J (2008) Multiple cardiovascular complications in a patient with Behcet’s disease. Korean J Intern Med 23(2):100–102
Black IW (2000) Spontaneous echo contrast: where there’s smoke there’s fire. Echocardiography 17(4):373–382
Yilmaz S, Karadag O, Yazisiz V, Altun B, Gezer M, Karaman M, Cinar M, Erdem H, Pay S, Dinc A (2013) Systemic involvements and preferred treatments in a large cohort of Behçet’s disease. Rheumatol Int 33(12):3025–3030
Kuno T, Tamura Y, Ono T, Murata M, Kuwana M, Satoh T, Fukuda K (2014) Recurrent right atrial thrombosis due to Behçet disease. Can J Cardiol 30(10):1250.e1–1250.e3
Efthimiou J, Johnston C, Spiro SG, Turner-Warwick M (1986) Pulmonary disease in Behçet’s syndrome. Q J Med 58(227):259–280
Acknowledgments
The patient’s consent was obtained to publicize the case. This study was supported by Nasonova Research Institute of Rheumatology (Moscow).
Conflict of interest
None.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Lisitsyna, T., Alekberova, Z., Ovcharov, P. et al. Left ventricular intracardiac thrombus in a patient with Behçet disease successfully treated with immunosuppressive agents without anticoagulation: a case report and review of the literature. Rheumatol Int 35, 1931–1935 (2015). https://doi.org/10.1007/s00296-015-3306-1
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00296-015-3306-1