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      Cardiac findings in patients with Behçet’s disease: Facts and controversies

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          Behçet’s disease (BD) is a systemic vasculitis of unknown etiology. It is characterized by recurrent oral and genital ulcers, uveitis, and a number of systemic manifestations. Although the disease is recognized worldwide, its prevalence is highly variable. A detailed review and analysis of the worldwide published reports on BD showed that not only the prevalence of the disease but also its cardiac complications differ according to the geographic distribution of the studied population. With the exception of France, Greece, Spain, and Italy, very few reports and studies regarding BD have been published from the western countries. Cardiovascular complications are an important cause of poor outcome in patients with BD. Over the past few years, many case reports and studies have been published, providing more facts about these complications. For example, peculiar echocardiographic findings in patients with aortic valve regurgitation and intracardiac thrombi secondary to BD were recently described. The role of these findings in the initial diagnosis of the disease, however, remains to be evaluated. On the other hand, some reports present contradictory results, especially concerning the left ventricular diastolic function, pathogenesis of coronary artery disease, and proper management of the cardiac complications in BD. Importantly, management of these complications is based mainly on the discretion of the treating physician due to the absence of large controlled studies and clear guidelines. This approach sometimes creates inconsistent data and allows controversies to persist. The review presented here will discuss some of the facts and controversies related to cardiac complications in BD.

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          Most cited references 48

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          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).

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            Behçet's disease--a contemporary review.

            Behçet's disease (BD) is a systemic vasculitis disorder of unknown etiology, characterized by relapsing episodes of oral aphthous ulcers, genital ulcers, skin lesions and ocular lesions. It can affect other systems including vascular, gastrointestinal and neurological systems. It occurs most frequently in an area that coincides with the Old Silk Route (between latitudes 30 degrees and 45 degrees north in Asia and Europe). BD is slightly more frequent and has a worse clinical course in men. It is believed to be due to an auto-immune process triggered by an infectious or environmental agent in a genetically predisposed individual. HLA-B51 is the most strongly associated risk factor. The International Study Group (ISG) for Behçet's Disease created a set of criteria for the diagnosis of BD. Available treatments include corticosteroids, azathioprine, cychlophosphamide, cyclosporine A, interferon-alpha, anti-tumour necrosis factor alpha agents, among others. BD has a variable course characterized by relapses and remissions. Prognosis depends on the clinical involvement. Loss of visual acuity and neurological disease are major causes of morbidity and disability.
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              Spectrum of cardiac lesions in Behçet disease: a series of 52 patients and review of the literature.

              Cardiac abnormalities in patients with Behçet disease (BD) include pericarditis, myocarditis, endocarditis with valvular regurgitation, intracardiac thrombosis, endomyocardial fibrosis, coronary arteritis with or without myocardial infarction, and aneurysms of the coronary arteries or sinus of Valsalva. Data regarding the clinical spectrum, prevalence, and outcome of cardiac lesions in BD are lacking. In this study, we report the main characteristics, treatment, and long-term outcomes of 52 patients with cardiac lesions from a cohort of 807 (6%) BD patients. Forty-five (86.5%) patients were male, with a mean (±SD) age at BD diagnosis of 29.3 ± 10.3 years.Cardiac involvement was the first feature of BD in 17 (32.7%) patients. Cardiac lesions included pericarditis (n = 20; 38.5%), endocarditis (mostly aortic insufficiency) (n = 14; 26.9%), intracardiac thrombosis (n = 10; 19.2%), myocardial infarction (n = 9; 17.3%), endomyocardial fibrosis (n = 4; 7.7%) and myocardial aneurysm (n = 1; 1.9%). Patients with cardiac involvement were more frequently male (86.5% vs. 64.9%; p < 0.01) and had more arterial (42.3% vs. 11.1%; p < 0.01) and venous lesions (59.6% vs. 35.8%; p < 0.01) compared to those without cardiac manifestations. Factors associated with complete remission of cardiac involvement were treatment regimens with oral anticoagulants, immunosuppressants, and colchicine. The 5-year survival rate was 83.6% and 95.8% (p = 0.03) in BD patients with and without cardiac involvement, respectively. After a median (Q1-Q3) follow-up of 3.0 (1.75-4.2) years, 8 patients had died, in 3 cases directly related to cardiac involvement.In conclusion, cardiac lesions affected 6% of our large cohort of BD patients. The prognosis of cardiac involvement in BD is poor and improves with oral anticoagulation, immunosuppressive therapy, and colchicine.

                Author and article information

                Anatol J Cardiol
                Anatol J Cardiol
                Anatolian Journal of Cardiology
                Kare Publishing (Turkey )
                July 2016
                : 16
                : 7
                : 529-533
                Department of Cardiovascular Medicine, Faculty of Medicine, Cairo University Hospitals; Cairo- Eygpt
                [* ]Department of Rheumatology and Rehabilitation, Faculty of Medicine, Cairo University Hospitals; Cairo- Eygpt
                [1 ]Department of Cardiology, West German Heart and Vascular Center, Essen University Hospital, Duisburg-Essen University; Essen- Germany
                Author notes
                Address for correspondence: Heba Farouk, MD, 18 El-Montasser Street, 12311, Agouza, Giza- Egypt Phone: +20 122 375 15 46 E-mail: Hfsaleh1@
                Copyright © 2016 Turkish Society of Cardiology

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License



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