https://doi.org/10.4081/reumatismo.2021.1369
Getting to the heart of the matter: diagnostic tools and therapeutic approach to cardiac involvement in Behçet syndrome A Tunisian case series
Submitted: 12 November 2020
Accepted: 23 February 2021
Published: 19 April 2021
Accepted: 23 February 2021
Abstract Views: 3679
PDF: 574
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All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Authors
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.
Saylan T. Life story of Dr. Hulusi Behçet. Yonsei Med J. 1997; 38: 327-32.
DOI: https://doi.org/10.3349/ymj.1997.38.6.327
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: https://doi.org/10.1007/s10067-015-3161-1
Eren H, Öcal L, Kalçik M, et al. Intracardiac thrombus in Behçet's disease. J Cardiovasc Echogr. 2016; 26: 22-4.
DOI: https://doi.org/10.4103/2211-4122.178472
Desbois AC, Wechsler B, Cluzel P, et al. (Cardiovascular involvement in Behçet's disease). Rev Med Interne. 2014; 35: 103-11.
DOI: https://doi.org/10.1016/j.revmed.2013.12.002
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: https://doi.org/10.1097/MD.0b013e3182428f49
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: https://doi.org/10.1016/j.echo.2009.07.025
Seyahi E, Ugurlu S, Cumali R, et al. Atherosclerosis in Behçet's syndrome. Semin Arthritis Rheum. 2008; 38: 1-12.
DOI: https://doi.org/10.1016/j.semarthrit.2007.09.009
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: https://doi.org/10.1016/j.ultrasmedbio.2017.12.010
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: https://doi.org/10.1136/annrheumdis-2018-213225
International Study Group for Behçet's Disease. Criteria for diagnosis of Behçet's disease. Lancet. 1990; 335: 1078-80.
DOI: https://doi.org/10.1016/0140-6736(90)92643-V
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: https://doi.org/10.14744/AnatolJCardiol.2016.7029
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: https://doi.org/10.1136/rmdopen-2020-001192
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.
DOI: https://doi.org/10.21470/1678-9741-2019-0003
Ehrlich GE. Vasculitis in Behçet's disease. Int Rev Immunol. 1997; 14: 81-8.
DOI: https://doi.org/10.3109/08830189709116846
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: https://doi.org/10.1016/j.ejim.2008.08.001
Demirelli S, Degirmenci H, Inci S, Arisoy A. Cardiac manifestations in Behcet's disease. Intractable Rare Dis Res. 2015; 4: 70-5.
DOI: https://doi.org/10.5582/irdr.2015.01007
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: https://doi.org/10.1155/2008/407972
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: https://doi.org/10.1016/S0046-8177(85)80250-1
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: https://doi.org/10.3349/ymj.1997.38.6.423
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: https://doi.org/10.1016/j.revmed.2006.07.019
Zhang Z, He F, Shi Y. Behcet's disease seen in China: analysis of 334 cases. Rheumatol Int. 2013; 33: 645-8.
DOI: https://doi.org/10.1007/s00296-012-2384-6
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: https://doi.org/10.1002/art.24149
Mogulkoc N, Burgess MI, Bishop PW. Intracardiac thrombus in Behçet's disease: a systematic review. Chest. 2000; 118: 479-87.
DOI: https://doi.org/10.1378/chest.118.2.479
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: https://doi.org/10.1053/ejvs.2000.1240
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: https://doi.org/10.4076/1752-1947-3-8869
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: https://doi.org/10.1007/BF02984606
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: https://doi.org/10.1097/MD.0000000000009102
Gürgün C, Ercan E, Ceyhan C, et al. Cardiovascular involvement in Behçet's disease. Jpn Heart J. 2002; 43: 389-98.
DOI: https://doi.org/10.1536/jhj.43.389
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: https://doi.org/10.1097/00005792-200301000-00006
Evereklioglu C. Current concepts in the etiology and treatment of Behçet disease. Surv Ophthalmol. 2005; 50: 297-350.
DOI: https://doi.org/10.1016/j.survophthal.2005.04.009
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: https://doi.org/10.1016/j.jsha.2013.02.001
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: https://doi.org/10.1186/1750-1172-7-20
Khalid U, Saleem T. Hughes-Stovin syndrome. Orphanet J Rare Dis. 2011; 6: 15-.
DOI: https://doi.org/10.1186/1750-1172-6-15
Chang SA. Tuberculous and infectious pericarditis. Cardiol Clin. 2017; 35: 615-22.
DOI: https://doi.org/10.1016/j.ccl.2017.07.013
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: https://doi.org/10.3904/kjim.2006.21.1.50
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: https://doi.org/10.17305/bjbms.2011.2625
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: https://doi.org/10.1016/j.jacc.2009.02.007
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: https://doi.org/10.2174/1874192400802010118
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: https://doi.org/10.1136/ard.56.3.205
Imazio M, Brucato A, Cemin R, et al. Colchicine for recurrent pericarditis (CORP): a randomized trial. Ann Intern Med. 2011; 155: 409-14.
DOI: https://doi.org/10.7326/0003-4819-155-7-201110040-00359
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: https://doi.org/10.1016/S0140-6736(01)05497-6
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: https://doi.org/10.1076/ocii.8.4.293.6453
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: https://doi.org/10.1016/j.clim.2018.08.004
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: https://doi.org/10.24953/turkjped.2019.04.016
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: https://doi.org/10.1016/S0248-8663(05)82284-2
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: https://doi.org/10.1097/MBC.0b013e328330bdc4
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: https://doi.org/10.1186/1477-9560-3-9
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> of the matter: diagnostic tools and therapeutic approach to cardiac involvement in Behçet syndrome A Tunisian case series. Reumatismo, 73(1), 32–43. https://doi.org/10.4081/reumatismo.2021.1369
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