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      Behçet’s Disease: an Insight from a Cardiologist’s Point of View

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          Behçet's disease (BD) is an enigmatic inflammatory disorder, with vasculitis (perivasculitis) underlying pathophysiology of its multisystemic affections. Venous pathology and thrombotic complications are hallmarks of BD. However, it has been increasingly recognized that cardiac involvement and arterial complications (aneurysms, pseudoaneurysms, rupture and thrombosis) are important part of the course of BD. Pericarditis, myocardial (diastolic and/or systolic dysfunction), valvular and coronary (thrombosis, aneurysms, rupture) involvement, intracardiac thrombi (predominantly right-sided) are, probably, the most frequent cardiac manifestations. Treatment of cardiovascular involvement in BD is largely empirical and aimed at suppression of vasculitis. The most challenging seems to be the treatment of arterial aneurysms and thromboses due to the associated risk of bleedings. Cardiologists should always bear in mind potential threats of (a)symptomatic cardiovascular involvement in BD.

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          Most cited references 46

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          Behçet's disease.

           T Sakane,  G Inaba,  M Takeno (1999)
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            Prevalence of Behçet's disease in Istanbul, Turkey.

            The prevalence of Behçet's disease (BD) is much higher in countries along the ancient Silk Route, extending from Japan to Mediterranean countries including Turkey, than in northern Europe and the USA. Three previous epidemiologic surveys have been carried out in different regions of Turkey. This study investigated the cross-sectional prevalence of BD in individuals aged > 12 years in Istanbul, Turkey, in two stages. The first stage aimed to identify individuals with recurrent oral ulcers (ROUs) by visiting them in their homes, and the second stage aimed to further examine those with ROUs for the presence of other BD-related manifestations under hospital conditions. The sample size was determined to be 24,000 with an expected BD prevalence rate of 1/1000 and a sampling error of 4/10,000, with a 95% confidence interval (CI) of 6-14/10,000. The number of individuals to be screened in each district was determined in proportion to the population of all districts in Istanbul. The standard questionnaire was applied to a total of 23,986 individuals at their homes. A history of ROU was recorded in 2289 individuals (9.5%), and a previous diagnosis of BD was recorded in 47. The diagnosis of ROU was confirmed in 700, and the diagnosis of BD was established in 101 according to the International Study Group criteria. The prevalence rate of BD was estimated as 42/10,000 (95% CI, 34-51/10,000) in Istanbul, Turkey. This survey conducted in Istanbul, the largest cosmopolitan city in Turkey with immigrants from all over the country, has a larger sample size than other previous studies, and therefore the reported prevalence rate of BD has a more acceptable confidence interval. This study aids in the estimation of the prevalence of BD in Turkey, and supports previous findings that Turkey has the highest prevalence rate of the disease in the world.
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              Clinical manifestations of Behçet's disease: an analysis of 2147 patients.

               A Boyvat,  A Gürler,  U Tursen (1997)
              To evaluate the prevalence of the clinical findings in Behçet's disease, we retrospectively analyzed the clinical data of 2147 Behçet patients from 9 to 87 years of age (mean age 38.3 years) followed up during the years 1976 through 1997. One thousand ninety three patients were male and 1054 patients were female. The male/female ratio was 1.03. The mean age at onset was 25.6 years. A family history of Behçet's disease was present in 7.3% of the patients. Positive pathergy was found in 1220 (56.8%) patients. All of the patients had mucocutaneous lesions. Out of the 2147 patients the disease manifested itself as only mucocutaneous involvement in 1168 patients. The prevalence of systemic manifestations was found as follows: 28.9% ocular involvement, 16.0% musculoskeletal involvement, 16.8% vascular involvement, 2.8% gastrointestinal involvement, 2.2% neurological involvement. Pulmonary involvement was seen in 20 (1.0%) patients, cardiac involvement was seen in 3 patients and renal involvement was observed in 2 patients. Male patients had vascular involvement 5.02, neurologic involvement 2.21 and ocular involvement 1.98 times more frequently than female patients.

                Author and article information

                Open Cardiovasc Med J
                The Open Cardiovascular Medicine Journal
                Bentham Open
                23 February 2010
                : 4
                : 63-70
                [1 ]Cardiology Office, CH-4310 Rheinfelden, Switzerland
                [2 ]Dudley Group of Hospitals NHS Trust, West Midlands, UK
                Author notes
                [* ]Address correspondence to this author at the Cardiology Office, POB 119, Marktgasse 10a, CH-4310 Rheinfelden, Switzerland Tel: +004161-832-45-55; Fax: +004161-833-97-56; E-mail: praxis@
                © Cocco and Gasparyan; Licensee Bentham Open.

                This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.


                Cardiovascular Medicine

                cardiovascular involvement, diagnosis, behçet's disease, treatment.


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