Getting to the heart of the matter: diagnostic tools and therapeutic approach to cardiac involvement in Behçet syndrome A Tunisian case series

  • A.S. Hammami | Department of Internal Medicine, Geriatric Unit, Hospital Fattouma Bourguiba of Monastir, Monastir, Tunisia; Biochemistry Laboratory, LR12ES05 LR-NAFS Nutrition - Functional Food and Vascular Health, Faculty of Medicine, University of Monastir, Monastir, Tunisia.
  • M. Jellazi Department of Internal Medicine, Geriatric Unit, Hospital Fattouma Bourguiba of Monastir, Monastir, Tunisia.
  • S. Arfa Department of Internal Medicine, Geriatric Unit, Hospital Fattouma Bourguiba of Monastir, Monastir, Tunisia.
  • S. Daada Department of Internal Medicine, Geriatric Unit, Hospital Fattouma Bourguiba of Monastir, Monastir, Tunisia.
  • K. Ben Hamda Department of Cardiology, Hospital Fattouma Bourguiba of Monastir, Monastir, Tunisia.
  • A. Achour Department of Radiology, Hospital Fattouma Bourguiba of Monastir, Monastir, Tunisia.
  • S. Ouali Department of Internal Medicine, Geriatric Unit, Hospital Fattouma Bourguiba of Monastir, Monastir, Tunisia; Biochemistry Laboratory, LR12ES05 LR-NAFS Nutrition - Functional Food and Vascular Health, Faculty of Medicine, University of Monastir, Monastir, Tunisia.


The aim was to investigate the frequency and spectrum of cardiac involvement (CI) in patients with Behçet syndrome (BS) in the Tunisian context, and to assess the clinical and imaging features, treatment, and outcomes. We retrospectively retrieved the medical records of patients with CI among 220 BS patients admitted to the hospital internal medicine department between February 2006 and April 2019, who fulfilled the International Study Group diagnostic criteria for BS. Ten patients (8 men, 2 women) were eligible for the study. Mean age was 37.3 years. Three patients had 2 isolated episodes of cardiac BS. The different types of CI were coronary artery disease (5/10), intracardiac thrombus (4/10), pericarditis (1/10), myocarditis (1/10), and myocardial fibrosis (1/10). Five patients had associated vascular involvement (50%). Medical treatment was based on corticosteroids and colchicine in all patients (100%), anticoagulants in 8 (80%), and cyclophosphamide followed by azathioprine in 9 (90%). The clinical course was favorable in 9 patients; 1 patient died. CI remains an important feature of BS because of its association with increased risk of mortality and morbidity. Therefore, early screening and detection with imaging methods are paramount. Also, better cooperation between rheumatologists and cardiologists could improve outcomes.



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Saylan T. Life story of Dr. Hulusi Behçet. Yonsei Med J. 1997; 38: 327-32. DOI:

Zhu YL, Wu QJ, Guo LL, Fet al. The clinical characteristics and outcome of intracardiac thrombus and aortic valvular involvement in Behçet's disease: an analysis of 20 cases. Clin Exp Rheumatol. 2012; 30: S40-5.

Wang H, Guo X, Tian Z, et al. Intracardiac thrombus in patients with Behcet's disease: clinical correlates, imaging features, and outcome: a retrospective, single-center experience. Clin Rheumatol. 2016; 35: 2501-7. DOI:

Eren H, Öcal L, Kalçik M, et al. Intracardiac thrombus in Behçet's disease. J Cardiovasc Echogr. 2016; 26: 22-4. DOI:

Desbois AC, Wechsler B, Cluzel P, et al. (Cardiovascular involvement in Behçet's disease). Rev Med Interne. 2014; 35: 103-11. DOI:

Geri G, Wechsler B, Thi Huong du L, et al. Spectrum of cardiac lesions in Behçet disease: a series of 52 patients and review of the literature. Medicine (Baltimore). 2012; 91: 25-34. DOI:

Han JK, Kim HK, Kim YJ, et al. Behçet's disease as a frequently unrecognized cause of aortic regurgitation: suggestive and misleading echocardiography findings. J Am Soc Echocardiogr. 2009; 22: 1269-74. DOI:

Seyahi E, Ugurlu S, Cumali R, et al. Atherosclerosis in Behçet's syndrome. Semin Arthritis Rheum. 2008; 38: 1-12. DOI:

Wechsler B, Du LT, Kieffer E. (Cardiovascular manifestations of Behçet's disease). Ann Med Interne (Paris). 1999; 150: 542-54.

Pu L, Li R, Xie J, et al. Characteristic echocardiographic manifestations of Behçet's disease. Ultrasound Med Biol. 2018; 44: 825-30. DOI:

Hatemi G, Christensen R, Bang D, et al. 2018 update of the EULAR recommendations for the management of Behçet's syndrome. Ann Rheum Dis. 2018; 77: 808-18. DOI:

International Study Group for Behçet's Disease. Criteria for diagnosis of Behçet's disease. Lancet. 1990; 335: 1078-80.

Behçet H, Matteson EL. On relapsing, aphthous ulcers of the mouth, eye and genitalia caused by a virus. 1937. Clin Exp Rheumatol. 2010; 28: S2-5.

Farouk H, Zayed HS, El-Chilali K. Cardiac findings in patients with Behçet's disease: Facts and controversies. Anatol J Cardiol. 2016; 16: 529-33. DOI:

Piga M, Floris A, Espinosa G, et al. Development and preliminary validation of the Behçet’s syndrome Overall Damage Index (BODI). RMD Open. 2020; 6: e001192. DOI:

Vural U, Kizilay M, Aglar AA. Coronary involvement in Behçet's disease: what are its risks and prognosis? (rare cases and literature review). Braz J Cardiovasc Surg. 2020; 34: 749-58.

Ehrlich GE. Vasculitis in Behçet's disease. Int Rev Immunol. 1997; 14: 81-8. DOI:

Harzallah O, Kerkeni A, Baati T, Mahjoub S. Oxidative stress: correlation with Behçet's disease duration, activity and severity. Eur J Intern Med. 2008; 19: 541-7. DOI:

Demirelli S, Degirmenci H, Inci S, Arisoy A. Cardiac manifestations in Behcet's disease. Intractable Rare Dis Res. 2015; 4: 70-5. DOI:

Kartal Durmazlar SP, Akgul A, Eskioglu F. Homocysteine may involve in the pathogenesis of Behcet's disease by inducing inflammation. Mediators Inflamm. 2008; 2008: 407972. DOI:

Lakhanpal S, Tani K, Lie JT, et al. Pathologic features of Behçet's syndrome: a review of Japanese autopsy registry data. Hum Pathol. 1985; 16: 790-5. DOI:

Gürler A, Boyvat A, Türsen U. Clinical manifestations of Behçet's disease: an analysis of 2147 patients. Yonsei Med J. 1997; 38: 423-7. DOI:

B'Chir Hamzaoui S, Harmel A, Bouslama K, et al. (Behçet's disease in Tunisia. Clinical study of 519 cases). Rev Med Interne. 2006; 27: 742-50. DOI:

Zhang Z, He F, Shi Y. Behcet's disease seen in China: analysis of 334 cases. Rheumatol Int. 2013; 33: 645-8. DOI:

Mahr A, Belarbi L, Wechsler B, et al. Population-based prevalence study of Behçet's disease: differences by ethnic origin and low variation by age at immigration. Arthritis Rheum. 2008; 58: 3951-9. DOI:

Mogulkoc N, Burgess MI, Bishop PW. Intracardiac thrombus in Behçet's disease: a systematic review. Chest. 2000; 118: 479-87. DOI:

Ozeren M, Mavioglu I, Dogan OV, Yucel E. Reoperation results of arterial involvement in Behçet's disease. Eur J Vasc Endovasc Surg. 2000; 20: 512-9. DOI:

Sokhanvar S, Karimi M, Esmaeil-Zadeh A. Recurrent acute myocardial infarction with coronary artery aneurysm in a patient with Behçet's disease: a case report. J Med Case Rep. 2009; 3: 8869. DOI:

Türkölmez S, Gökçora N, Alkan M, Gorer MA. Evaluation of myocardial perfusion in patients with Behçet's disease. Ann Nucl Med. 2005; 19: 201-6. DOI:

Ma W, Liang Y, Zhu J. Ten-year progress of coronary artery lesions prior to Behçet disease diagnosis: A case report and care-compliant article. Medicine (Baltimore). 2017; 96: e9102. DOI:

Gürgün C, Ercan E, Ceyhan C, et al. Cardiovascular involvement in Behçet's disease. Jpn Heart J. 2002; 43: 389-98. DOI:

Kural-Seyahi E, Fresko I, Seyahi N, et al. The long-term mortality and morbidity of Behçet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore). 2003; 82: 60-76. DOI:

Evereklioglu C. Current concepts in the etiology and treatment of Behçet disease. Surv Ophthalmol. 2005; 50: 297-350. DOI:

Ghori MA, Al Sousi A, Al Mahmeed W, et al. A case report of a right ventricular mass in a patient with Behçet's disease: Myxoma or thrombus? J Saudi Heart Assoc. 2013; 25: 85-9. DOI:

Emmungil H, Yaşar Bilge N, Küçükşahin O, et al. A rare but serious manifestation of Behçet's disease: intracardiac thrombus in 22 patients. Clin Exp Rheumatol. 2014; 32: S87-92.

Saadoun D, Wechsler B. Behçet's disease. Orphanet J Rare Dis. 2012; 7: 20. DOI:

Khalid U, Saleem T. Hughes-Stovin syndrome. Orphanet J Rare Dis. 2011; 6: 15-. DOI:

Chang SA. Tuberculous and infectious pericarditis. Cardiol Clin. 2017; 35: 615-22. DOI:

Bletry O, Mohattane A, Wechsler B, et al. (Cardiac involvement in Behçet's disease. 12 cases). Presse Med. 1988; 17: 2388-91.

Kwon CM, Lee SH, Kim JH, et al. A case of Behçet's disease with pericarditis, thrombotic thrombocytopenic purpura, deep vein thrombosis and coronary artery pseudo aneurysm. Korean J Intern Med. 2006; 21: 50-6. DOI:

Gao N, Han W, Ci WP, et al. (Clinical data analysis of cardiovascular involvement in Behcet's disease). Zhonghua Yi Xue Za Zhi. 2016; 96: 1523-6.

Zivkovic M, Zlatanovic M, Zlatanovic G, et al. Anticardiolipin antibodies in patients with Behcet's disease. Bosn J Basic Med Sci. 2011; 11: 58-61. DOI:

Higashihara M, Mori M, Takeuchi A, et al. Myocarditis in Behcet's disease--a case report and review of the literature. J Rheumatol. 1982; 9: 630-3.

Okcun B, Baran T, Babalik E, Kücükoglu S. Multichamber masses and constrictive pericarditis in Behçet's disease. Clin Exp Rheumatol. 2003; 21: S55.

Rose NR, Neumann DA, Herskowitz A. Coxsackievirus myocarditis. Adv Intern Med. 1992; 37: 411-29.

Friedrich MG, Sechtem U, Schulz-Menger J, et al. Cardiovascular magnetic resonance in myocarditis: A JACC White Paper. J Am Coll Cardiol. 2009; 53: 1475-87. DOI:

Sonia H, Khaldoun BH, Sylvia M, et al. Stenosis and aneurysm of coronary arteries in a patient with Behçet's Disease. Open Cardiovasc Med J. 2008; 2: 118-20. DOI:

Antman EM, Anbe DT, Armstrong PW, et al. ACC/AHA guidelines for the management of patients with ST-elevation myocardial infarction; A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee to Revise the 1999 Guidelines for the Management of patients with acute myocardial infarction). J Am Coll Cardiol. 2004; 44: E1-e211.

Huong DLT, Wechsler B, Papo T, et al. Endomyocardial fibrosis in Behçet’s disease. Ann Rheum Dis. 1997; 56: 205-8. DOI:

Imazio M, Brucato A, Cemin R, et al. Colchicine for recurrent pericarditis (CORP): a randomized trial. Ann Intern Med. 2011; 155: 409-14. DOI:

Sfikakis PP, Theodossiadis PG, Katsiari CG, et al. Effect of infliximab on sight-threatening panuveitis in Behçet's disease. Lancet. 2001; 358: 295-6. DOI:

Wechsler B, Bodaghi B, Huong DL, et al. Efficacy of interferon alfa-2a in severe and refractory uveitis associated with Behçet's disease. Ocul Immunol Inflamm. 2000; 8: 293-301. DOI:

Desbois AC, Biard L, Addimanda O, 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. DOI:

Isıyel E, Bakkaloğlu S, Oğuz D, et al. An adolescent case of extensive Behçet`s disease successfully treated with Infliximab. Turk J Pediatr. 2019; 61: 585-8. DOI:

Assaad-Khalil S, Sobhy M, Abou-Seif M, El-Sawy M. C 37 Cardiac manifestations of Behçet's disease: clinical, genetic and echocardiographic study. Rev Méd Int. 1993; 14: 42s. DOI:

Pamuk GE, Pamuk ON, Orüm H, et al. Might platelet-leucocyte complexes be playing a role in major vascular involvement of Behçet's disease? A comparative study. Blood Coagul Fibrinolysis. 2010; 21: 113-7. DOI:

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

Hammami S, Mahjoub S, Ben-Hamda K, et al. Intracardiac thrombus in Behçet's disease: two case reports. Thromb J. 2005; 3: 9. DOI:

Behçet syndrome, cardiovascular system, coronary artery disease, myocarditis, immunosuppressive agents.
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How to Cite
Hammami, A., Jellazi, M., Arfa, S., Daada, S., Ben Hamda , K., Achour, A., & Ouali, S. (2021). Getting to the <em>heart</em&gt; of the matter: diagnostic tools and therapeutic approach to cardiac involvement in Behçet syndrome A Tunisian case series. Reumatismo, 73(1), 32-43.